Sickle cell anaemia (SCA) is the commonest inherited haemoglobinopathy in Nigeria and is associated with high morbidity and mortality, particularly in early childhood in most of the affected population. The cardiac manifestations of SCA are a significant feature of the disease but there is a paucity of information on the cardiovascular involvement in SCA in Nigeria and Africa. The size of the sickle cell problem in the country is growing rapidly and there should therefore be a greater awareness of the cardiac problems associated with SCA. This review highlights the clinical features, changes in cardiac structure and function at rest and on exercise, cardiac pathology and associated heart diseases in SCA as well as the recent global progress made in the understanding of the cardiovascular changes in the disease. Emphasis is laid on data derived from Nigerian studies.