BACKGROUND: Children with hydrocephalus represent a heterogeneous group with various aetiologies and disability profiles. Over the years, continuous changes in medical care have occurred and updated information is important. AIM: To study disability profiles in aetiological and gestational age subgroups of children with hydrocephalus in the 1990s. METHOD: A population-based series of 114 children, 70 with infantile hydrocephalus and 44 with hydrocephalus associated with MMC. All the children were examined clinically and interviewed. RESULTS: Learning disabilities were present in 47 % of children with infantile hydrocephalus compared with 16 % of those with MMC, cerebral palsy in 27 % vs. zero and epilepsy in 34 vs. 11 %. Even after excluding children with cerebral palsy, the majority had abnormal tendon reflexes and scored below the 5th centile on a motor test. Hydrocephalus overt at birth, low gestational age, a perinatal origin, enlarged ventricles at follow-up and several shunt revisions all indicated risk factors for a poor outcome. CONCLUSIONS: In spite of major advances in management, hydrocephalus in children still has a considerable impact on outcome. Being born very preterm and with a hydrocephalus that is already overt at birth involve the highest risk of a poor outcome. Apart from major impairments, the children frequently have definite motor problems.