HighlightsThis is a rare inheritable condition which results in raised total HCG levels.Common causes of raised HCG need to be excluded prior to considering this syndrome.A diagnosis can prevent unnecessary surgical or oncological treatment.

Epithelioid trophoblastic tumor is a rare and distinctive pathologic entity within the complex family of gestational trophoblastic disease. We describe a case of epithelioid trophoblastic tumor occurring in a 34-year-old woman, who presented with a large uterine tumor 3 years following an uncomplicated pregnancy. The clinicopathologic findings in this case are typical of this unusual entity and consistent with current literature, with the exception of negative beta-human chorionic gonadotrophin levels. The distinguishing features from other intermediate trophoblastic tumors and tumor-like lesions are discussed.