Klippel-Trénaunay syndrome is a rare congenital condition that classically presents as a triad of varicosities, bone or soft-tissue hypertrophy, and cutaneous hemangiomas. The authors describe a case of this disease in an otherwise healthy patient with unilateral leg edema and intermittent bleeding from prominent ankle varicosities. The diagnosis was made on the basis of clinical presentation, diagnostic studies, and a comprehensive literature review. An overview of the pathology, treatment, and prognosis of this disease is provided.