A rare case of penile epithelioid sarcoma in a 26-year-old white man is presented. The patient had a firm, slow growing, painless mass associated with penile deviation on erection, which was initially misdiagnosed as Peyronie's disease 5 years previously. With the onset of decreased urinary stream the lesion was biopsied, with a resultant diagnosis of epithelioid sarcoma. The patient underwent radical penectomy and perineal urethrostomy, and received 6,000 cGy. external beam radiotherapy postoperatively. Two years postoperatively the patient remained free of clinical disease. We review the pathophysiology, clinical characteristics and treatment of penile epithelioid sarcoma.