Hepatic hemangioendothelioma is the most frequent hepatic tumor in infancy, but rarely detected in adults. This tumor can cause vascular lesions that can act as arteriovenous fistulas and produce life-threatening high output congestive heart failure with respiratory compromise. We report a case of a 35 years-old woman who developed nausea, vomiting, weight lost and abdominal mass in which the pathological examination of the hepatic lesion showed a infantile hepatic hamangioendothelioma. This is the third case in adult patients described in the literature.