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Myxoid liposarcoma (MLS) is the most commonly encountered liposarcoma subgroup, accounting for about 50% of all cases. Metastatic MLS of the heart is extremely rare. Herein we describe for the first time metastasis of MLS to the left atrium and left upper pulmonary vein in a 54-year-old woman who was admitted with shortness of breath and cough persisting for 2 weeks. The patient reported that a total surgical excision of MLS of the left thigh followed by radiotherapy was performed 4 years ago. An emergency operation was performed due to rapidly progressive worsening of clinical condition and echocardiographic determination of left atrial mass protruding into the left ventricle and obstructing the mitral inflow throughout the diastole. The mass could not be totally excised because it was tightly adhered to the surrounding tissue. Postoperative magnetic resonance imaging (MRI) showed a 5× 3 cm residual tumor deforming the posterior wall of the left atrium entirely and extending into the left upper pulmonary vein. Histopathological examination was consistent with MLS. In conclusion, considering probable cardiac metastasis in patients presenting with respiratory symptoms with medical history of soft tissue sarcomas would be life saving. The case is discussed, and a review of the literature in relation to the metastatic involvement of the heart by MLS is presented.