Primary cardiac tumors are uncommon, with an autopsy frequency of 0.001%-0.28%: 75% of them are benign, and 50-75% of these are myxomas. Often the first neurological manifestations of a cardiac myxoma include transient ischemic attacks and ischemic strokes. Although thrombolytic therapy represents the gold standard for acute ischemic stroke treatment, its safety and effectiveness in stroke patients with myxoma is unknown. From the analysis of the literature on thrombolysis in ischemic stroke patients with myxoma we report clinical evidence supporting the use of thrombolytics, and the application of thrombolysis in this setting of patients. 23 previous reports presented patients with acute ischemic stroke and associated cardiac myxoma treated with thrombolytic therapy: 16 patients were treated with intravenous thrombolysis, 4 patients with intra-arterial thrombolysis and 3 patients were treated with bridging therapy (intravenous alteplase followed by local mechanical thrombolysis). Our review showed that the possible risk of major bleeding in these patients resulted in limited and small hemorrhages; furthermore patients who developed cerebral hemorrhage did not deteriorate clinically: waiting for further confirmation and additional data from a future register, these observations may suggest that, notwithstanding a possible publication bias, i.v. thrombolytic therapy may be a safe treatment in these patients.