About 7 years ago, we undertook the resection on a patient with a tumour of the wall of the left ventricle near the atrioventricular junction. The pathologic diagnosis after the operation was peripheral primitive neuroectodermal tumour (pPNET) of the left ventricular wall. Microscopically the cell had a small, round, deeply basophilic nucleus, rich in chromatin, and little or no surrounding perikaryon. To our knowledge, no article has ever reported a case of primary myocardial PNET which grew towards the pericardial cavity and there is no adequate data on optimal treatment of PNET in the heart at present. In our case since the tumour recurred in situ and had no evidence of distant metastases, we considered orthotopic cardiac transplantation.