A 49-year-old man with Lutembacher's syndrome associated with frontal meningioma referred to our hospital. He also suffered from exertional dyspnea. Transthoracic echocardiography demonstrated mitral valve area of 1.48 cm2, moderate mitral stenosis, and left atrial dimension (LAD) of 5.6 cm with no clot. TEE revealed severe mitral stenosis, mitral valve area of 1.05 cm2 with wilkins 8-10 score, ejection fraction of 50%, and enlarged left atrium (LAD = 5.8 cm) with no clot. Induction of anesthesia was commenced taking into account the patient's specific circumstances, which meant the risk of surgery was high. During surgery, the mitral valve was replaced and the atrial septal defect was repaired without a patch. This case underscores the significance of the adoption of an appropriate therapeutic strategy in the treatment of Lutembacher's syndrome with meningioma before meningioma surgery.