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Background: Myxoma is the most common type of primary cardiac tumor. The clinical signs and symptoms include nonspecific systemic manifestations, mechanical interference with cardiac function, and embolization. The neurological complications include embolic infarction, neoplastic aneurysm, hemorrhage and metastasis. Case: We report a patient with cardiac myxoma that manifested as acute vertigo. Neurological examination showed gaze-evoked nystagmus and imbalance. Magnetic resonance imaging revealed multiple small infarctions in the cerebellum. Conclusion: Acute vertigo can be an initial manifestation of ardiac myxoma.