OBJECTIVE: To study the outcome in children with brain tumours diagnosed in the first year of life, we followed up 27 consecutive children who were diagnosed between 1980 and 2005 in a single institution. METHODS: Tumour control and neurological, endocrine and cognitive complications and their impact on behavioural and emotional adjustment and health-related quality of life (HRQoL) were comprehensively assessed in 11 survivors (mean follow-up time 12.3 years). RESULTS: Persistent neurological complications occurred in 9/11 patients, endocrine and growth complications in 4/11, and cognitive deficits leading to school problems/impaired choice of occupation in 8/10. Behavioural and psychological adjustment problems were reported by 4/6 patients and 7/10 parents. HRQoL as rated by patients and their parents was considerably lower than that of healthy controls. In comparison with healthy controls, social functioning was rated by the patients and the parents as the QoL dimension most affected. HRQoL was lowest for patients with high-grade tumour histology and more intense therapy. CONCLUSION: Long-term survivors of brain tumours diagnosed in the first year of life are not only at great risk of neurological and cognitive complications, but also of social isolation thereby substantially decreasing self-rated HRQoL.