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Fibromyxosarcoma is common in head and neck, vessel, omentum, and reproductive system, with low-grade malignant behavior. However, primary atrial fibromyxosarcoma with highly malignant behavior is extremely rare.A 34-year-old female presented with oppression in the chest, short breath, and onset of headache as initial symptoms. The preoperative echocardiogram showed a medium-size echogenic mass close to the posterior leaflet of the mitral valve in the left atrium.Primary atrial fibromyxosarcoma with multiple-system metastases.The patient underwent surgery, and the tumor was removed completely. The diagnosis of left atrium fibromyxosarcoma was confirmed through postoperative histopathological examination. Positron emission tomography/computed tomography scan was performed, which revealed multiple metastases to left adnexa, bilateral adrenal glands, left iliacus, right lateral ventricle, and skeletal system.The patient died of cerebral hernia caused by hemorrhage from the metastatic brain tumor, 30 days after the surgery, without receiving chemotherapy or radiotherapy.Cardiac fibromyxosarcoma is a rare primary malignant cardiac neoplasm, probably with systemic metastases. The possibility of malignancy should be considered as differential diagnosis for cardiac mass.