We aim to review the imaging findings of Klippel-Trenaunay syndrome. This disorder characterized clinically by a triad of capillary malformations manifesting as a port-wine stain, venous varicosities typically along the lateral aspect of the lower extremities, and bone and/or soft tissue hypertrophy. Imaging of extremities shows unilateral venous varicosities, persistent embryonic veins, anomalous of the superficial and deep venous system, low-flow venolymphatic malformations, and bony and soft tissue hypertrophy. Other findings include neurospinal as cavernoma, aneurysm, and hemimegalencephaly, pulmonary as pulmonary thromboembolism and pulmonary hypertension and visceral as gastrointestinal and genitourinary vascular anomalies. Imaging may detect associated lesions and differentiate from simulating lesions.