The authors report a case of Hailey-Hailey disease or familial benign chronic pemphigus and review the literature concerning the surgical treatments used for this disabling disease. The majority of authors recommend excision followed by split skin graft, while others prefer excision followed by directed healing or simple excision-suture. The case reported here is the first reported case of cure of Hailey-Hailey disease combined with mammaplasty in a single stage.