During a routine physical exam, an 18-day-old male infant was noted to have persistent abdominal distention and prominent vascular markings over his abdominal wall. Laboratory studies were significant for an elevated alpha-fetoprotein level of 7051 ng/ml and mild anemia. Abdominal ultrasound and CT scan demonstrated a large, heterogeneous mass in the lateral segment of the left lobe of the liver. Although the patient did not have congestive heart failure or coagulopathy, surgical resection was performed to rule out malignancy. Histopathologic examination revealed a type I infantile hemangioendothelioma. This case report reviews the presentation and treatment of infantile hemangioendotheliomas and the differential diagnosis of neonatal hepatic neoplasms.