Background and Objective: Severe neonatal anaemia can impair cerebral oxygen supply. Data on long-term outcomes following severe neonatal anaemia are scarce. Methods: Clinical data and neurodevelopmental outcome of 49 (near) term infants with haemoglobin concentration after birth <6.0 mmol/l were retrospectively collected and analysed. In a subgroup of 28 patients, amplitude-integrated EEG was available and in 25 infants cerebral MRI was obtained. Infants were followed up at 14–35 months of age and assessed with the Griffiths Scale of Mental Development or Bayley Scale of Infant Development. Results: Eighteen patients (37%) died during the neonatal period. In 25 patients MRI was per-formed. A predominant pattern of injury on MRI was seen in the basal ganglia and thalami in 7 patients (28%), whereas some form of white matter injury was present in 16 (64%) and a combination in 3 (12%). Follow-up data were available for 26 patients (84% of survivors). Formal assessment of neu-rodevelopmental outcome was performed in 20 of 31 (65%) infants who survived (median age: 19 months, range: 14–35 Sixteen infants (80%) had a developmental quotient appro-priate for age in the first 2 years after birth. On motor out-come, 1 patient (5%) scored below average (Z-score –1.10). One patient developed cerebral palsy. Conclusion: Early neurodevelopmental outcome in surviving patients with se-vere neonatal anaemia was within the normal range in the majority of the survivors. MRI showed mild-to-moderate white matter injury in two thirds of the infants. Prospective-ly collected data with a longer follow-up period are needed. Neonatal anaemia has a diverse aetiology, and it can cause an acute life-threatening situation through hypovo-laemic shock and hypoxia at birth, as well as through mul-tiple organ failure in the first days of life. During hypovo-laemic shock, redistribution of blood flow occurs and may be preferentially directed to the brain, which possi-bly prevents more severe adverse neurological sequelae. Anaemia may result in encephalopathy, which can be as-sessed using aEEG (amplitude-integrated EEG).Data on MRI findings and neurodevelopmental out-come of survivors of neonatal anaemia are limited. This article is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes as well as any dis-tribution of modified material requires written permission.severe anaemia is often associated with perinatal asphyx-ia, it is hard to distinguish with neuro-imaging whether cerebral injury is due to anaemia or the associated hypox-ia-ischaemia.The aim of this retrospective study is to describe aEEG and MRI findings following severe neonatal anaemia in (near) term infants admitted at a level III neonatal inten-sive care unit, and relate these findings to neurodevelop-mental outcome at approximately 2 years of age.We retrospectively collected clinical data from medical records of patients who presented with severe anaemia in the neonatal in-tensive care unit of the VU Medical Center, Amsterdam, or the Wilhelmina Children’s Hospital, University Medical Center, Utrecht, between January 2000 and June 2011.Patients with an initial haemoglobin (Hb) concentration <6.0 mmol/l ([mg/dl] = [mmol/l] ×1.61) and a gestational age ≥36 weeks were eligible for this study. Infants with chromosomal ab-normalities or inborn errors of metabolism were excluded. In 9 patients, the first Hb concentration was measured after a blood transfusion had already been administered immediately af-ter birth. Initial Hb concentrations were therefore estimated, based on the fact that a blood transfusion of 20 ml/kg increases Hb con-centration by approximately 3.0 mmol/l. These estimated values were used as the initial Hb concentrations. Various clinical data of vital organ failure were collected, in-cluding the need for respiratory or circulatory support and the aEEG background pattern. MRI in the VU Medical Center was performed on a 1.5-tesla magnet (Siemens Vision, Erlangen, Germany). The MRI protocol included T1, T2 and diffusion-weighted images (DWI). In the Wil-helmina Children’s Hospital, the same sequences were obtained on a 1.5-tesla MRI, and more recently on a 3-tesla MRI (ACS-NT Sys-tem and Achieva, respectively; Philips Medical Systems, Best, the Netherlands). We assessed predefined brain areas on both conventional im-aging and DWI.