Aggressive angiomyxoma is a rare, locally aggressive tumor and occurs exclusively in the pelvic and perineal regions of women. But this tumor can occur in men, and the ratio of man to woman is about 1:6. We report a case of aggressive angiomyxoma of the scrotum in a 75-year-old man. The resected mass measured 23 21 10 cm and weighed 1,150 g, and was covered with thick but intact scrotal skin. The cut surface of the mass showed pale yellow to grayish white loose and edematous fibrous tissue with focal cystic degeneration. Microscopically, the mass was composed of loosely arranged bland-looking spindle or stellate cells and variable sized blood vessels with thickened and hyalinized wall. The spindle cells were strong positive for vimentin, focal, weak positive for desmin and smooth muscle actin but negative for S-100 protein. The spindle cells were also positive for estrogen and progesterone receptors.