BACKGROUND Epithelioid hemangioendothelioma is a malignant mesenchymal tumor of unknown etiology. They tend to be asymptomatic or with non-specific symptoms. The lesion is usually multiple and variable size. METHODS We describe a clinical case of a 23-years-old patient diagnosed with a pelvic mass, a possible uterine fibroid or adnexal mass, and multiple liver lesions that seemed an advanced ovarian cancer presentation and after liver biopsy turned out to be a hepatic epithelioid hemangioendothelioma. CONCLUSIONS It may be confused with a metastatic process in diagnostic imaging. There have been described some possible risk factors but the etiology remains unknown. The prognosis is usually lethal in 50% of cases. The surgical removal of the lesion and liver transplant appear to be the only hope for these patients. CONCLUSIONS Epithelioid hemangioendothelioma must be part of our differential diagnosis when we find a liver tumour, especially in young women. Treatment is excision of the tumour in limited disease. In the case of unresectable disease are candidates for liver transplantation.