Background: Sickle cell disease (SCD) is a global lifelong but preventable genetic blood disorder and it is most prevalent in sub-Saharan Africa within populations of very similar geographic distribution to malaria. Objective: To determine the prevalence and patterns of sickle cell disease in children attending tertiary and non- tertiary hospitals in Imo State, Nigeria. Methods: The study was a hospital based retrospective cross sectional survey from year 2004 to 2013 that identified all children with sickle cell disease that attended hospital. Data was collected using a proforma from patients‟ medical records. Descriptive analyses were done with frequencies and summary statistics. Chi square statistics were computed to determine significant relationships with the p set at<0.05. Results: The prevalence rate of sickle cell disease was 5%. The most common symptom was fever (87.6%) followed by bone pain (45%) with the complaint of general body weakness appearing to be significantly associated with the type of health care institution attended. (p=0.000).The most common clinical sign was jaundice (59.8%), followed by pallor (51.1%); and the clinical complication experienced the most was infection (50.3%), followed by vaso-occlusive crises (36%). The occurrence of haemolytic crises appeared to be significantly associated with the type of health care institution attended (p=0.004). Conclusion: Infection appears to be a leading complication and a trigger to subsequent sickle cell events within our environment. Therefore the management intervention should emphasis on infection prevention strategies within a framework of a preventive- interventional approach.