Proximal-type epithelioid sarcoma is an uncommon but aggressive soft-tissue malignancy involving proximal body parts especially perineum and genitalia of adult patients. This entity is a variant form of the conventional epithelioid sarcoma, which is frequently encountered at extremities of adolescents or young adults. The authors present a case of proximal-type epithelioid sarcoma of the scrotum with comprehensive staging and monitoring with PET/CT.