We present a case of pelvic aggressive angiomyxoma presenting as urinary retention in a male. A 46-year-old male presented with urinary retention and was found on computed tomography (CT) scan of the pelvis to have a large pelvic tumor. A transrectal ultrasound guided needle biopsy of the tumor and prostate revealed a myxoid tumor; low volume, low grade prostate cancer was also detected. The patient underwent radical prostatectomy and excision of the pelvic tumor which was diagnosed as aggressive angiomyxoma (AAM). The patient was free of recurrence after 1 year of follow up. AAM is a benign myxoid tumor seen very rarely in males. Treatment consists of surgical excision with negative margins. Tumors variably express estrogen and progesterone receptors. Immunohistochemistry should be used to exclude other benign and malignant tumors. Patients should be followed with axial imaging as recurrence is common.