Klippel-Trenaunay-Weber syndrome is characterized by a triad of features, namely, vascular nevi, venous varicosities, and hyperplasia of hard and soft tissues in the affected area. Involvement of the orofacial region is uncommon, but nevertheless, a wide range of orofacial abnormalities may necessitate specialized dental and anesthesia management. We have documented the manifestations in 2 affected persons, outlined the nosology, reviewed the literature, and tabulated craniofacial anomalies and orodental complications. Special emphasis is given to the hematologic factors (bleeding tendencies) and vascular involvement (hemangiomata) which may significantly influence orodental management and anesthesia.