– The reported case concerns an unusual localisation of “Royal Tumour” connected with von Recklinghausen's neurofibromatosis. Surgery was necessary because of the increasing difficulty of the patient in having sexual relations due to the progressive swelling of the penile neoformation. The typical clinical picture, the integrity of the corpora cavernosa and the negative impact of the neoformation on the patient's sexual life all indicated the necessity for operation. Results were satisfactory both surgically and functionally/aesthetically and the patient regained normal sexual activity.