A 28-year-old man was admitted because of dyspnea on effort. His tricuspid valve had been affected by granulocytic sarcoma and manifested tricuspid valve stenosis 8 years previously. After chemotherapy and radiation therapy, the tumor had disappeared and the tricuspid valve stenosis was relieved. Echocardiography showed that the posterior leaflet of the mitral valve was affected by the tumor, and Doppler ultrasonography revealed mild mitral valve stenosis. Biopsy of the anterior chest wall detected granulocytic sarcoma. Chemotherapy was started. The tumor size was reduced and the mitral valve stenosis became slight. Primary cardiac granulocytic sarcoma is very rare and stenosis of the atrioventricular valve by relapse of this tumor after complete remission is extremely unusual.

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