Hailey-Hailey disease is a rare hereditary dermatosis that begins in the 2nd or 3rd decade of life. The skin lesion is characterized by a localized, recurrent eruption of small vesicles on an erythematous base. It courses remissions and exacerbations. It seldom begins in early childhood, and main treatment modalities are conservative ones.

We report a case of Hailey-Hailey disease that began on a 7-month old infant and improved by surgical treatment. In according to review of the previous reports, it is probably the earliest onset age and it is may be the first case which was treated with surgery in Korea.