MPNST is defined as nerve sheath tumors arising from a peripheral nerve, or malignant tumors converting from benign neurogenic tumors.[1,3] About half of cases happen to occur along with neurofibromatosis type 1 (NF-1), which is a genetic mutation on the 17th chromosome.[1] As a result of the varying nomenclature in the past and different diagnostic criteria, few cases of cardiac MPNST have been reported. This evidence concerns the gene NF1 and nerve sheath neoplasm.