Two young infants were found to have choledochal cyst. In the first patient, the only clinical manifestation was asymptomatic hepatosplenomegaly. She developed bilious ascites after a percutaneous liver biopsy. The second patient presented with fluctuating jaundice and spontaneous perforation of the cyst resulting in bilious ascites. Particularly in infants, symptomatology of the choledochal cyst is extremely variable. A high index of suspicion will lead to an aggressive diagnostic approach and avoidance of serious sequelae.