Phacomatosis pigmentovascularis is a rare genodermatosis characterized by a combination of melanocytic nevi and vascular malformations. A new type of phacomatosis pigmentovascularis was recently described which included cutis marmorata telangiectatica congenita and aberrant Mongolian spots and was named type V. We report a 3-month-old girl with diagnosis of phacomatosis pigmentovascularis type V.