Prior to the advent of effective medical therapies, the only treatment option for patients with idiopathic pulmonary arterial hypertension (IPAH) was lung transplantation. We present the case of a woman who underwent single-lung transplantation for the treatment of IPAH > 10 years ago in whom chronic rejection developed. Despite complete obliteration of the allograft, it was noted that her PA pressure levels had almost normalized. Therefore, an allograft pneumonectomy was performed. To our knowledge, this is the first reported case of the regression of pulmonary vascular disease following lung transplantation with subsequent successful removal of the allograft.