Aggressive angiomyxoma denotes an extremely infrequent mesenchymal tumour. In virtually every case it involves genital, pelvic or perineal female structures. Cases involving male patients are extremely rare. It is a distinctive tumour with a characteristic clinical course and specific and well characterized microscopic features. The authors report an additional clinical case in a young male patient with massive scrotal and penile involvement, necessitating exeresis followed by complex reconstructive procedure implying flap and graft use. A review of the available literature concerning etiopathogenic, clinical, imagiologic, histological and differential diagnosis, therapeutic and prognostic aspects is also presented.