PURPOSE: To investigate the usefulness of carbon dioxide digital subtraction angiography (CO2-DSA) in direct puncture sclerotherapy of venous hemangiomas involving extremities. MATERIALS AND METHODS: Direct puncture sclerotherapy was performed in 12 patients with venous hemangioma of extremities. After direct puncture of the hemangiomas, 30-50 cc of CO2 was manually injected and digital subtraction angiograms were obtained. If draining vein was visualized, a tourniquet was applied at the proximal site to compress the draining veins. There after, radiopaque embolic materials such as 1:3 mixture of Lipiodol and absolute ethanol or ethanolamineoleate-Lipiodol-Avitene (microfibrillar collagen hemostat) mixture were slowly injected under the fluoroscopic guidance. RESULTS: Rapid injection of large amount of a CO2 enabled the visualization of wide areas of venous hemangiomas in 11 patients. Draining veins were reliably demonstrated in 10 patients and pulmonary embolism of embolic materials was effectively prevented by proximal tourniquet application. Because of radiolucent nature of CO2 retained in hemangiomas, we could clearly identify the distribution of radiopaque embolic materials under fluoroscopy. Retained CO2 also could be used as a guide for additional multiple puncture of hemangiomas. There was no systemic symptoms or complications related to CO2-DSA. CONCLUSION: CO2-DSA is a convenient, safe and useful angiographic technique in direct puncture sclerotherapy of hemangiomas involving extremities.