Clinical Key message
Portal Hypertension is not a classical presentation of Caroli’s
Syndrome. However, some young children can present with overt signs and
symptoms indicative of advanced disease state despite the improvement in
imaging technology which has decreased its diagnostic age. High index of
clinical suspicion can help in timely diagnosis and management.
Keywords : Caroli’s Syndrome; Hematemesis; Pediatrics; Portal
Hypertension.