Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by capillary vascular malformation and pigmentary nevus but with a wide variability in clinical presentation. A case of a 9-month-old patient is reported, who presented with capillary malformation and hemihypertrophy. These features typically are seen in Klippel–Trenaunay (KT) syndrome, a syndromic type of congenital vascular malformation. However, in addition, this child had large persistent Mongolian spots, the presence of which allowed us to classify this case as PPV, type II. This is a condition with a clinical course not always benign as KT syndrome
