Cardiac myxoma is a rare but curable cause of ischemic stroke. Current guidelines do not address the use of intravenous thrombolysis for embolic stroke caused by cardiac myxoma. The risk of hemorrhage due to occult tumor emboli or microaneurysms is a major concern. We describe a 45-year-old man who had an embolic stroke in the left middle cerebral artery. The initial National Institutes of Health Stroke Scale (NIHSS) score was 16. He received intravenous thrombolysis 2 h and 52 min after stroke onset. No intracranial hemorrhage developed. A cardiac mass was found in the left atrium and removed surgically 84 h after stroke. Pathological study showed a myxoma with extensive hemorrhage and thrombus over the surface. At the 3-month follow-up, the NIHSS score was 9 and the modified Rankin scale score was 3. Our experience with this patient supports the hypothesis that intravenous thrombolysis may be safely used in the treatment of embolic stroke due to cardiac myxoma.