Acne inversa is a chronic inflammatory disease of the hair follicles. It can lead to severe functional and psychological impairment. It is characterized by inflamed painful nodules, abscesses, fistulas and scarring in late stages of the disease. The causes of acne inversa are still not fully understood. Conservative treatment options such as antibiotics may lead to clinical improvement; however they do not produce healing. Therapy of choice, especially in severe forms, is radical wide excision of all affected areas. Despite a variety of treatment options, acne inversa is still a therapeutic challenge.