We describe a 16-year-old female with Nevus comedonicus (NC) that has been developed since birth. The lesions had a unilateral, linear or zosteriform distribution on the left lower leg and posterior thigh, and were well closely arranged, slightly depressed papules that had dark firm plugs resembling comedones in their centers. In the meantime, recurrent infections with fistula, abscess and residual scars had been complicated. Histopathologic features showed deep invagination of epidermis and dilated follicular orifices filled with keratin plugs in the centers of papules.