Cerebrovascular disease (CVD) is one of the most clinically devastating complications in children with sickle cell anaemia (SCA) and an important cause of death in this population.To determine the incidence of CVD in children with SCA and to characterise the risk factors and other related clinical and imaging aspects.We conducted a retrospective, cross sectional, descriptive study of paediatric patients with SCA and CVD that covered the period between January 1992 and February 2002.A total of 131 children with SCA were registered in the period between January 1992 and February 2002, during which five children suffered some kind of CVD event. This figure corresponds to 3.81% of the patients with SCA and 7.69% of the total number of children with CVD. The most frequent clinical presentation of the CVD is bouts of seizures and the most frequently observed type of CVD is ischemic, chiefly located in the middle cerebral artery. Of the different predictors that exist for ischemic CVD, haemoglobin count was found to be lower than in haemorrhagic CVD.CVD is a common complication in the progress of patients with SCA with a high percentage of neurological sequelae.