Klippel–Trenaunay syndrome is a rare but well-documented congenital malformation. Klippel–Trenaunay syndrome has sometimes been used interchangeably with Klippel–Trenaunay–Weber syndrome. However, Klippel–Trenaunay syndrome is the correct term used for the triad of congenital anomalies. Klippel–Trenaunay–Weber syndrome or Parkes-Weber syndrome is accepted as a separate entity consisting of the triad of Klippel–Trenaunay syndrome accompanied by a clinically apparent arteriovenous fistula. Hemodynamically insignificant arteriovenous malformations do not preclude a diagnosis of Klippel–Trenaunay syndrome. It is important to differentiate between the 2 syndromes because treatment and prognosis are so different. Parkes-Weber syndrome has a poor prognosis for limb viability. This article describes a case study of an infant presenting with Klippel–Trenaunay including a review of the syndrome and treatment recommendations.