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AbstractA malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft-tissue sarcoma of ectomesenchymal origin. The World Health Organisation created the term MPNST to replace previous terminology such as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and myxofibrosarcoma for tumours of neurogenic origin with similar biological behavior.[1–3] The vast majority of these tumours develop in extremities. They also tend to be located in unusual sites of the body, such as the pelvic retroperitoneum, infratemporal fossa, intrapericardium, and mediastinum.[1,3,4] This case study presents a patient with an extremely rare primary cardiac MPNST..