Objective: To review the clinicopathologic features of the infantile hepatic hemangioendothelioma (IHHE) in adults, so as to improve the diagnosis and treatment for this disease. Methods: The clinical data of 7 adult patients (age ranged from 32 to 63 years) with pathological confirmation of IHHE, who underwent surgical treatment from January 2003 to December 2013 in Eastern Hepatobiliary Surgery Hospital, were retrospectively analyzed. Results: Of the 7 patients, 5 cases had no obvious clinical symptoms before operation, and the other cases had intermittent pain in the right upper abdomen and anorexia, respectively. The routine blood tests and liver function parameters showed no obvious abnormality, tumor markers and hepatitis virus indexes were all negative, and results of the CD34 immunohistochemical staining were all positive. All cases had a solitary lesion, and diagnosis was not confirmed by preoperative imaging examination. All patients were followed up regularly after operation, and no tumor recurrence was noted, as of August 2014. Conclusion: IHHE in adults is an extremely rare condition, and in cases without a history of hepatitis or positive result of tumor markers, and after exclusion of cavernous hemangioma by CT and MRI findings, this condition should be considered.