Congenital absence of the penis is a very rare malformation. The incidence is about 1:30 million persons or 1:16,000 necropsies. The cause of the malformation is unknown. Frequent associated malformations of the lungs, the colon and the urinary tract are determinating the prognosis. The therapy of choice in case of isolated penile agenesis is primary orchidectomy, later vaginoplasty and hormonal feminisation. We report our experience with two cases.