2.1. Background: Perivascular epithelioid cell tumors (PEComas) of the uterus encompass a rare family of mesenchymal neoplasms characterized by co-expression of smooth muscle and melanocytic markers in epithelioid or spindle cells. Due to their unclear malignant potential, a close follow-up after surgical resection is mandatory. Case Presentation:We present the case report of a 55-yearold woman who underwent hysterectomy due to the diagnosis of a uterus myomatosus with growing tendency. Preoperative imaging by ultrasonography and magnetic resonance imaging scan (MRI) did not show specific findings. A uterine PEComa was diagnosed by morphology and immunohistology. Conclusion:Uterine PEComas are extremely rare tumors, which, in most cases, are diagnosed incidentally based on specific morphological and immunohistochemical features.