Background Sickle cell anaemia (SCA) is a leading cause of child morbidity and mortality affecting 4% of all newborns in Uganda. Cerebrovascular accident (CVA) is a serious complication of SCA in childhood and 10% of patients with SCA will have had a CVA by the age of 20 years. Hitherto there has been limited information on SCA-related stroke in Uganda.

Objective To describe the clinical presentation of children with SCA-related stroke; and haematological indices and interventions in those with and without CVA.

Method We studied 184 SCA children aged 2–18 years attending the sickle cell services of Mulago Hospital in Uganda from February to June 2011. Forty six children with CVA were compared with 138 without CVA. Analysis was done using chi square and Fisher’s exact test.

Results There were 184 SCA patients: 46 with CVA and 138 without CVA. The median age at first CVA was 4 years [Inter-quartile range (IQR) 2–6]. Symptoms of children with SCA-related stroke included behaviour changes, headache and seizures. Findings on physical examination included hemiplegia (37), aphasia (10), and limb ataxia (16). Four of the 46 children with SCA-related stroke had an HbS <30%, versus 0/138 without CVA (p = 0.004). Children with SCA-related stroke (44/46) were more likely to have received a blood transfusion than those without CVA (99/138): OR 8.6 (95% CI 2.0, 37.5).

Conclusion Cerebrovascular accident in SCA patients is a serious and devastating complication. Patients presented with several neurological signs and symptoms. Further studies to elucidate the outcome and management are urgently needed.