Sickle cell disease (SCD) is a family of inherited blood disorders of variable severity which have in common haemolytic anaemia, recurrent painful crises, end-organ failure and the risk of reduced life expectancy. In Britain, the condition predominantly occurs among families of African or Afro-Caribbean origin. This study examines the effects of the condition on the psychological adjustment and family functioning of 39 children with SCD and 24 control children. Participants were assessed on measures of behaviour, depression, self-esteem, IQ and reading skills, family relationships and maternal mental health. Assessment on the Family Environment Scale showed that SCD children came from families who reported more cohesion and less conflict than did the families of controls. Maternal mental health showed no significant differences between the SCD group and controls. Children with SCD showed an IQ deficit of five points, a difference that was not statistically significant. However, a meta-analysis of six studies in the literature, including this one, did show a highly significantly decreased intellectual ability. SCD children did not show significant differences from controls on measures of depression and self-esteem. However, differences in behavioural problems were found between the three groups, with the SC group showing more behavioural problems. Results of regression analyses suggest that maternal mental health is associated with children's behavioural problems.