Soft tissue sarcomas constitute about 1% of all malignancies registered among adult population. In Poland its incidence is less than 1.000 new cases per year. They can occur in any anatomical locality, with approximately 20% found in the peritoneal cavity. One of them is an extremely rare mezynchemal tumor originating from epithelioid tissues, i.e. perivascular epithelioid cell tumor (PEComa). It was first reported in 1991 and in 1996 the name PEComa was proposed for the tumors that originate from the perivascular epithelioid cells. So far 100 cases of PEComa have been described, with 1/3 of them located in the uterus. In most reports the disease follows a mild course, although there are also reports on its recurrence after several years from initial detection. Additionally, the reports emphasize difficulties to differentiate them from leiomyosarcomas. The article presents a rare case of PEComa located in the uterine corpus in a 26-year-old patient. Case study: A 26-year-old woman operated on for USG findings and increased βhcg with suspected ectopic pregnancy. USG scan revealed hyperechogenic mass, 4cm in diameter, well vascularized with low resistance, with hypoechogenic space of 5.8mm in diameter. No gestational sack was seen inside the uterus. The ovaries were normal on USG. No free fluid was visualized in the abdominal cavity. A mass of 4cm in diameter located in the right round ligament of the uterus, with firm margins, covered by the peritoneum was totally enucleated by laparoscopy. Sum-up: PEComa is a rare soft tissue tumor often originating from the uterus. In some atypical uterine tumors gynecologist should consider its occurrence and plan the surgery accordingly.