Introduction: Proximal-type epithelioid sarcoma (PES) represents an extremely rare and aggressive form of soft tissue neoplasm, typically presenting as a painless subcutaneous nodule in the trunk often located in the genital area. Case report: A 46-year-old female was subjected to the excision of a growing soft tissue mass in the mons pubis that, at histology, was identified as PES. The tumor showed an extreme aggressiveness involving subsequently adjoining structures and lymph nodes despite subsequent wide surgical resections during the following months. Discussion: Gynecologists should pay careful attention to all soft tissue masses of the perineal area or external genitalia. It is important to know the possible genital localization of PES which, although rare, is an aggressive high-grade soft tissue tumor with a deceitful behavior, poorly sensitive to chemotherapy and radiotherapy. Surgery, though wide and demolitive, often fails to obtain the necessary radicality.