Myxoma is the most common and prevalent type of tumor in the heart, and 75% of tumors occur in the left artrium. However, the incidence of artrial myxoma complicated with frontoparietal cavernous malformation is extremely low, which has been rarely reported. Here, we reported a male 53-year-old patient diagnosed with left artrial myxoma complicated with left frontoparietal cavernous malformation. Whether the myxoma or the cavernous malformation should be firstly managed is challenging. Considering the complexity of these complications, a two-stage therapeutic regimen is chosen. First, the left artrial myxoma was surgically resected, followed by appropriate interventions for the left frontoparietal cavernous malformation. No evident postoperative complications occurred.The patient was successfully discharged.