We report a case of acantholytic dyskeratotic epidermal nevus in a 28-year-old female. The patient showed unilateral, asymptomatic, grouped, 2 mm sized, brownish, follicular ant non-follicular, keratotic papules disposed along Blaschko´s lines on the right side of th back, flank and abdomen. She had had this condition for 4 years. The biopsy specimen obtaine, from the abdomen showed hyperkeratosis, parakeratosis, acanthosis, acantholytic suprabasal clefts, corps ronds, and grains. Although cryotherapy with liquid nitrogen was per formed on half of the lesions, the whole skin lesions healed with focal hypertrophic scars 4 weeks later. There was no recurrence after a 12 months follow-up period. To our knowledge this is the first reported case of acantholytic dyskeratotic epidermal nevus in Korean dermatologi, literature.