The case of a 13-year-old girl with primary Epstein-Barr virus (EBV) infection and concomitant cholestatic hepatitis, which initially presented as acute acalculous cholecystitis (AAC), is described. The diagnosis of AAC was documented by clinical and ultrasonographic criteria, whereas acute EBV infection was confirmed serologically. AAC may develop during the course of acute EBV infection, especially in patients with cholestatic hepatitis.