Choledochal cysts are relatively rare and are an uncommon cause of cholestasis. Early diagnosis and prompt treatment can prevent complications such as cholangitis, cirrhosis and portal hypertension. This article reviews a rarely reported disease in Africans in whom only 3 cases were documented over the 18 year period in Nigeria. The 3 cases were all females with ages between less than a month and 13 months at presentation. All presented with abdominal swelling with or without jaundice or acholic stools. The use of real-time ultrasonography antenatally and postnatally aided the diagnosis in our patients. Two of the patients presented and were operated and both made full recovery confirming the importance of early surgical intervention. The third patient died, and exemplified the consequences of delayed diagnosis and treatment which occur not uncommonly in developing countries mostly because of sparse and or expensive tertiary health care facilities.