Klippel-Trenaunay syndrome (KTS) is a rare disease and a syndrome that is characterized by the triad of congenital port wine stains,venous malformations with or without varicose veins, bone or soft tissue hypertrophy. Lymphatic filariasis is a filarial disease which usually occurs with genital anomalies, pathologies associated with lymphedema or elephantiasis caused by a filarial infectious agent. In this case report, a 20-year-old male patient admitted to our clinic for diagnosis and treatment with right leg edema and lesions compatible with dermatological manifestations. In the list of differential diagnosis, filarial elephantiasis was also thought, consequently KTS was diagnosed. (Turkiye Parazitol Derg 2012; 36: 188-90).