Conradi-Hünermann-Happle syndrome is an X-linked dominant chondrodysplasia punctata occurs only in girls because it is usually lethal in hemizygous males. The clinical manifestations include an ichthyosiform scaling erythroderma at birth that usually resolves during the first year of life and is replaced by bands of follicular atrophoderma, linear hyperkeratosis, and pigmentary abnormalities. Cataracts, congenital scarring alopecia, saddle-nose deformity, short stature, asymmetric limb reduction defects, and stippled calcifications of the epiphyses on radiographs can be seen. This patient is 13-years-old girl presented for localized congenital scarring alopecia involving the scalp. O/E, there were pitted scar-like follicular atrophoderma involving the dorsal surface of the hands and band-like linear atrophoderma on the trunk. Her nose is saddle-shaped and the mother gave history that her daughter had diffuse red scaly rash since birth and disappeared later.