Aggressive angiomyxoma (AA) is an uncommon tumor occurring in females and is rarely reported in males with propensity to recur. Due to its presence in perineal and genital region, it has to be differentiated from other myxoid neoplasms. The tumor expresses estrogen and progesterone receptors, which may have a role to play in tumor therapy. Wide local excision remains the mainstay of the treatment. We present a case of AA excised from left spermatic cord.