We report the case of a 46-year-old woman who had undergone cardiac transplantation for a malignant, right ventricular sarcoma. Five years later, she experienced pulmonary hypertension and a pulmonary artery aneurysm. Medical management of the pulmonary hypertension being unsuccessful, she underwent surgical exploration of the pulmonary artery aneurysm and bilateral pulmonary endarterectomy. Intra-operative findings revealed pulmonary artery sarcoma and an unresectable pulmonary artery aneurysm. Copyright (C) 2004 by the International Society for Heart and Lung Transplantation.