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.
© 2020 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.