Malignant peripheral nerve sheath tumor (MPNST) is a very rare sarcoma of the heart, and few cases have been reported. Herein, we retrospectively reviewed clinical manifestations, imaging features and management of our patient and other reported cases. A 32-year-old woman was referred to the emergency department of our institution with expiratory dyspnea, edema of face for a month. The patient was initially diagnosed with asthma at a local hospital based on a history of fatigue, cough and expiratory dyspnea, as well as negative electroencephalogram (ECG) and chest radiography. Based on computed tomography (CT) and cardiac magnetic resonance imaging (CMRI) in our hospital, she was found to have a malignant tumor involving right atrium. The tumor was diagnosed as MPNST according to histopathological results. The tumor was deemed unresectable during the surgery. Then, the patient was referred for chemotherapy and radiotherapy. The patient deteriorated and died 4 months later. Cardiac MPNST is very uncommon with nonspecific clinical and imaging characteristics according to limited cased reported. CMR, due to the high tissue resolution and multiple sequence imaging advantages, is useful for the detection, location and evaluation whether there is involvement of adjacent structures, and may help better clinical decision-making.