Background: Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumors that present in infancy. This study was designed to review clinical experiences with IHHE and to suggest the management strategies. Methods: The medical records of 23 patients (M:F=10:13) with IHHE treated at Asan Medical Center between 1996 and 2009 were analyzed retrospectively. Results: Median age at presentation was 38 days (1 day-381 days). Sonographically detected fetal liver masses led to diagnosis of IHHE in 7 patients (30%). 5 patients (22%) were diagnosed with IHHE incidentally without suspicious symptoms. Other presenting features were congestive heart failure (n=5, 22%), skin hemangiomata (n=3, 13%), abnormal liver function tests (n=2, 9%), and hepatomegaly (n=1, 4%). All patients were diagnosed with IHHE via imaging studies such as ultrasonography, CT and MRI, and 3 of them were confirmed to have IHHE by histopathology. Thirteen patients had a unilobar single tumor and 10 had bilobar multifocal disease. Four patients showed elevated levels of serum alpha-fetoprotein at presentation, 3 of whom had multiple bilobar disease. One patient with congestive heart failure and a resectable unilobar tumor underwent surgical resection. Three patients with congestive heart failure and unresectable tumors were managed with hepatic artery embolization with or without medical treatment, and all of them showed significant decreases in tumor size. Five patients were treated with interferon, 8 patients with steroids, and 6 patients were observed without any treatment. Clinical courses and treatment outcomes were not significantly different between medically treated and non-treated groups. Overall, complete disappearance of tumor or a greater than 90% decrease in tumor size was noted in 8 patients, a 50 to 90% decrease in 7 patients, a less than 50% decrease in 4 patients, and no significant change in 1 patient. No patient died of tumor. Conclusion: IHHE had a benign clinical course with low morbidity and mortality. IHHE could be managed conservatively with or without medical treatment, and surgical approach should be limited to the patients with intractable symptoms such as heart failure or consumptive coagulopathy. For surgically unresectable case with significant symptoms, hepatic artery embolization or medical treatment could be used.