CONCLUSIONS
A definitive diagnosis of Caroli syndrome is only possible through CT or MRCP or histological examination of the liver specimen, which remains a challenge in the resource-limited settings. It is important to consider Caroli’s syndrome in the differential diagnosis in patients presenting with features of portal hypertension. Delay in treatment can lead to severe life threatening hematemesis, cholangitis or even cancer. Slow and silent progression of the disease warrants regular follow-up.