A 21-year-old Korean male was referred to our department in June, 1999 for the evaluation of extensive reddish patches and gray-bluish pigmentation since birth. Physical examination revealed two kinds of patches over the various parts of the body. Reddish patches suggesting nevus flammeus were located on the left side of face, chest, and both upper and lower extremities. Gray-bluish pigmented patches suggesting nevus of Ota were found on both the periorbital areas. A 0.5×0.6cm erythematous papule was found on the right anterior chest. The results of its histopathologic examination were compatible with pyogenic granuloma. We made the diagnosis of phakomatosis pigmentovascularis associated with pyogenic granuloma as well as Sturge-Weber syndrome and Klippel-Trenanunay syndrome.