Introduction: Choledochal cysts (CCs) are congenital dilatations of the intrahepatic and/or extrahepatic bile ducts. Type IVa cysts involve both ductal systems and account for 15–35% of cases. The classic triad of abdominal pain, jaundice, and palpable abdominal mass is seen in only a minority of patients. Case presentation: A previously healthy 3-year-old girl presented with hematemesis andmelena, without prior abdominal pain or jaundice. Examination revealed mild jaundice, pallor, and firm hepatosplenomegaly without ascites. Laboratory tests showed elevated transaminases, conjugated hyperbilirubinemia, and raised ALP and GGT levels, with preserved synthetic liver function. Abdominal ultrasonography demonstrated dilatation of the intrahepatic ducts and common bile duct (CBD), coarse hepatic echotexture, and splenomegaly . Contrast-enhanced computed tomography confirmed fusiform CBD dilatation measuring 10.9 × 6 × 5.6 cm with associated intrahepatic ductal dilatation and irregular liver surface. Upper gastrointestinal endoscopy revealed Grade III esophageal varices. A diagnosis of Type IVa choledochal cyst complicated by portal hypertension was made. After resuscitation and endoscopic variceal band ligation, complete cyst excision with Roux-en- Y hepaticojejunostomy was performed. The postoperative course was uneventful, with marked clinical and biochemical improvement. Repeat upper GI endoscopy showed improvement of the varices, and banding was performed. Conclusion: This case underscores the importance of considering Type IVa choledochal cyst in children presenting with portal hypertension, even in the absence of the classic triad of abdominal pain, jaundice, and a palpable abdominal mass. Early diagnosis and timely surgical correction are critical for achieving a favorable prognosis.