Abstract
Phakomatosis pigmentovascularis was first described in 1947 as a distinctive association of cutaneous hemangioma and pigmentary nevi by Ota et al.
We describe a 7-year-old boy who, since birth, had three kinds of discolored patches over the various parts of the body: blue spots (dermal melanocytic nevi), reticulated reddish patches (nevus flammeus), and hypopigmented macules (nevus anemicus). No systemic disease was found.