RESUMO
Aggressive angiomyxoma is an uncommon neoplasm that preferentially involves the pelvic and perianal regions of reproductive age females. These tumors are rare in men and merit a wider recognition in male urologic pathology. We report a case of a 65 year old male who presented with a swelling in the left perineal region since 2 years which gradually increased in size to 10 x 10 x 8 cms. CT scan revealed a well defined lobulated heterogenous minimally enhancing mass in the left ischiorectal fossa extending upto the subcutaneous plane. Microscopically stellate to spindle shaped cells arranged in a loose myxoid stroma with numerous thin to thick walled vessels were seen. There was no evidence of pleomorphism, hyperchromatism or mitotic activity. This case is reported because of its rarity in males. It is a benign tumor and requires a wide excisional surgery.