Phacomatosis pigmentovascularis(PPV) is a rare congenital capillary malformation syndrome in association with pigmented skin lesions. According to Hasegawa’s classification, it is categorized into five subtypes and each one is further subdivided into (a) or (b) depending on the absence or presence of systemic manifestations, respectively. Among them, the most common type is PPV II which is characterized by a coexistence of nevus flammeus and aberrant Mogolian spot. We evaluated a 17-month-old female infant, born full-term through cesarean delivery, weighing 4.4kg. Apgar score was 9 at first and 10 at fifth minutes. She presented with port-wine stains on her whole body including face and aberrant Mongolian spots on the trunk and extremities. In addition, Nevi of Ota involving both sclerae were also noted. There was no history of seizure. Laboratory exams including neonatal screening test were normal. Magnetic resonance imaging and ultrasonography of the brain showed no remarkable findings. Furthermore, the ophthalmic examination revealed no glaucoma or other associated problems. The infant was diagnosed as type IIa PPV, or Phacomatosis cesioflammea defined by Hapel’s new classification in 2005. We report this uncommon case of inborn vascular malformation syndrome.