We report a rare case of atypical epithelioid hemangioma of the penis in a 42-year-old man with a painful penile dorsal nodule, first misdiagnosed on simple penile ultrasonography as Peyronie's disease. Even though the patient underwent treatment, the lesion improved and a penile echo-color Doppler scan showed abundant vascularization. After local excision, the pathologic diagnosis was atypical epithelioid hemangioma (central epithelioid cells bordered by immature vessels), with immunohistochemical positivity for CD31 and CD34 and negativity for epithelial membrane antigen. At 12 months after surgery, the patient was free of disease. Penile echo-color Doppler ultrasonography is essential to perform a differential diagnosis between Peyronie's disease from other penile nodular diseases.