The perivascular epithelioid cell tumors (PEComas) mimic rare epithelial mesenchymal tumors with epithelioid features and perivascular clear cells that co-express the muscle and melanocytic markers. The PEComas include lymphangioleiomyomatosis, clear cell sugar tumor, angiomyolipoma, and myomelanocytic tumors. We report a case involving 37-year-old woman with a PEComa in the left parametrium. The patient was referred to our hospital with an adnexal tumor detected during medical examination. Therefore, she underwent laparoscopic mass excision surgery. Immunohistochemical staining of the tumor cells indicated positivity for Melan A, HMB45, and desmin. Finally, the tumor was diagnosed as a PEComa. The patient is undergoing regular follow-up, and no recurrence has been noted at 2 years after the surgery.