The Authors reports a case of Verneuil's disease in a perineal location. They focus on etiopathogenetic aspects and problems of differential diagnosis, before reviewing the various therapeutic options. Surgery is the only effective therapy and takes the form of an extensive excision of the cutaneous zone affected with 2nd intention recovery, or a dermoepidermal auto-graft. A long follow-up is important to monitor the possible recurrence of lesions generally due to the incomplete removal of the area affected by the process.