Balanitis xerotica obliterans (BXO), first described by Stuhmer in 1928, is a chronic, progressive, atrophic, sclerosing process involving prepuce, glans and urethra. Its aetiology is unknown. After a short terminological excursus and a review of the aetiological hypothesis, we have focused on BXO in daily urological practice. We are here describing the clinical presentation and its differential diagnosis with premalignant and malignant lesions of the penis. We tried to define the relationship between BXO and squamous cell carcinoma of the penis. Particular attention was then cast on urethral stenosis. Finally, we focused on the treatment of BXO: corticosteroid local therapy as first line treatment or as adjuvant therapy after circumcision, surgical therapy including circumcision, laser therapy of the glans lesions, meatoplasty in the stenosis of the anterior urethra. We also stressed the need for histological examination of the preputial specimen for a correct follow-up and for medicolegal reasons.