Background: The spectrum and incidence of congenital heart diseases amongst African population are not well defined in literature. There is the need to further elucidate the spectrum and epidemiology of congenital heart diseases amongst African. Aim: This study was conducted to highlight the spectrum and occurrence of congenital heart diseases in an African population. Methodology: All paediatric patients who were autopsied over a 98-month period were checked for occurrence of congenital heart diseases. Attempts were made to ascertain the primary disease and causes of death. We noted their bio-data, ages, sexes and causes of death. The results were analysed with SPSS version 17. The results formed the basis for discussion and recommendations. Results: There were 135 cardiac related deaths and 36 of them were in paediatric age brackets. The mean age was 4.7 + 1.08 months with a standard deviation of 6.27. M:F ratio was 1:1.1. The commonest anomaly was ventricular septal defect. There was a weak association with other congenital anomalies. The commonest cause of death was bronchopneumonia. Discussion and Recommendation: The disease pattern showed reasonable similarity with reports from other parts of the world. However, there is need for development of capacity for prenatal diagnosis of congenital heart diseases in Africa. This will widen the net of diagnosis and improve accuracy of incidence studies in Africa.