Spontaneous (nontraumatic, noninfectious) rupture and/or dissection of the pulmonary trunk or secondary pulmonary arteries in the setting of pulmonary hypertension is rarely considered as a cause of sudden death. Systemic-to-pulmonary vascular shunts and rheumatic mitral stenosis have been the most common predisposing factors, occurrence in association with recurrent thromboembolic hypertension rarely having been documented. Recently, we had the opportunity to study a 52-year-old white woman with catheterization-proved "primary" pulmonary hypertension who died suddenly with cardiac tamponade from rupture/dissection of the pulmonary trunk, and who had morphological evidence of extensive thromboembolic pulmonary vascular disease.