Klippel-Trenaunay syndrome (KTS) is a rare complex vascular with limb hypertrophy. KTS diagnosed if at least two of the three features capillary malformation, venous and soft tissue and/or bone overgrowth are present. Of these, malformation (i.e., port-wine stain) most commonly observed feature but may be absent in atypical KTS. We herein report case unilateral