Phakomatosis pigmentovascularis is a rare cutaneous malformation characterized by a combination of nevus flammeus and pigmentary nevus such as nevus of Ota, nevus of Ito, mongolian spots, nevus spilus. It is often associated with systemic organ involvement. We report two cases of phakomatosis pigmentovascularis. The first was a 11-year-old boy, who had had diffuse nevus flammeus, mongolian spots since birth, and congenital glaucoma. The second case was a 9-year-old boy who had had nevus flammeus, mongolian spots, nevus of Ota, and nevus of Ito over various parts of his body since birth. He also had leg-length discrepancy, avascular necrosis of the femur head, congenital temporal alopecia and varicose veins. (Korean J Dermatol 1998;36(3): 506-510)