Obstruction of major pulmonary vessels with organized thromboemboli is a rare sequelae of acute pulmonary embolic disease. Depending on the extent and duration of vascular occlusion, patients experiencing this unusual disorder may develop significant pulmonary hypertension and cor pulmonale. If left untreated, the ultimate clinical outcome is right heart failure and death. Over the past several decades, the description of this clinical entity has evolved from an autopsy curiosity to a recognized cause of chronic pulmonary hypertension. Also, during this same time period, surgical capabilities have greatly advanced, providing these patients a potentially life-saving remedy for this debilitating form of pulmonary vascular disease. This article provides a historical perspective for our current understanding of major vessel chronic thromboembolic pulmonary hypertension as a distinct clinical disorder. It also chronicles the developments in surgical techniques that have made thromboendarterectomy of the pulmonary arterial bed a reality.