Background:Klippel-Tranuanay Syndrome is a rare sporadic disease characterized by clinical triad of capillary malformation; soft tissue and bony hypertrophy; and atypical varicosity. Although this syndrome was first described more than hundred years ago, exact incidence has not been estimated yet. Several theories have been postulated to describe its pathogenesis. Clinical presentation of this syndrome is protean ranging from minimal asymptomatic disease to life threatening bleeding and embolism. Management of this syndrome includes careful diagnosis, prevention and treatment of complications.Main observation:We are presenting a case of Klippel-Tranuanay Syndrome in a 19-year-old male patient presenting with varicosity in lateral aspect of right leg. On careful examination other components of the syndrome were found. Patient was kept on yearly follow up.Conclusion:Usually patients with Klippel-Tranuanay Syndrome present with complication. This case shows that there is also a need for medical solutions in patients who seek advise for cosmetic reasons.