Abstract
Klippel-Trenaunay syndrome (KTS) is characterized by the triad of cutaneous capillary malformations (port-wine stains), asymmetrical disturbed growth of soft tissues and/or bone, and venous and lymphatic malformations. Prolonged and recurrent cutaneous bleeding from ulceration of the capillary and/or venous and/or lymphatic malformation can be life-threatening. We report for the first time to our knowledge a case of Klippel-Trenaunay syndrome treated by oral sirolimus.