Hepatic involvement is common in acute Epstein-Barr virus (EBV) infection in children. It usually manifests as a transitory elevation of transaminases in up to 80% to 90% of patients, and they normalize by 2 to 6 weeks. A cholestatic pattern with elevated gamma-glutamyl transferase (γGT) and alkaline phosphatase (ALP) is common, in up to 60% in young adults. However, jaundice is very rare occurring in only 5% of pediatric patients. We report here an 8-month-old female with EBV infection who developed obstructive jaundice 2 weeks after the initial infection. Radiologic investigations were compatible with choledochal cyst type IVa complicated by stone formation in the common bile duct. In case of clinical exacerbation or nonamelioration of liver function tests in EVB infection, another diagnosis should be addressed. This highlights the importance of close follow-up in these patients in order not to miss a serious underlying condition such as choledochal malformation.