Myxoma of the left ventricle is exceedingly rare and to the best of our knowledge not a single case of its recurrence has been reported in Japan. We have recently experienced a case in which a myxomatous tumor of the left ventricle recurred at the same site as the primary lesion 2.5 years after operation and was treated by surgical excision. The patient was a 28-year-old female who, under the diagnosis of myxoma of the left ventricle, underwent surgical removal of the tumor and mitral valve replacement at her age of 25 years. Although her postoperative course was uneventful, she was noticed, at her age of 28 years, of her inaudible prosthetic valve clicks on auscultation at the outpatient service. Echocardiography revealed a tumor mass in the left ventricle, which tended to grow with the elapse of time. Echocardiography on rehospitalization disclosed a mobile cystic tumor on the posterior wall of the left ventricle, while pulmonary arteriography also revealed a movable tumor in the left ventricle. Intraoperatively, there was noted a solid tumor, composed partly of cystic structure, on the posterior wall of the left ventricle and quick pathology led to a suspected diagnosis of myxoma. Since the tumor was found to have involved the ventricular septum and myocardial tissue of the posterior wall of the left ventricle, its complete surgical excision was impossible. The tumor, with its growth pattern and morphology, was diagnosed as a malignant clinical behavior one, although histopathological evidence indicates its benignancy.