Phakomatosis pigmentovascularis is characterized by the coexistence of pigmentary and vascular nevi, which are occasionally associated with systemic organ involvement. We report a 12-year-old male, who showed bilateral nevus of Ota on the forehead, periorbital area, sclera and soft palate, nevus of Ito on the scapular area, mongolian spot on the sacral area and widespread nevus flammeus. He was also associated with congenital temporal alopecia on the lower portion of left temporal region since birth. (Kor J Dermatol 29(2):252 255)