Acne inversa is a rare chronic and debilitating inflammatory skin disease. The authors report a case of a 45-year old male who presented with acne inversa in the inguinal, perineal, and scrotal areas. After unsatisfactory pharmacological treatment a wide surgical excision of the affected skin was performed in stages. On follow-up the patient presented with a very good cosmetic and functional result. A review of the most recent literature is also presented.