Pulmonary capillary hemangiomatosis (PCH) is a rare and an only recently described vascular disease. As one of the causes of primary pulmonary hypertension, the need for the establishment of helpful diagnostic radiological criteria for this condition has become imperative. However, the unstable hemodynamic condition of patients with primary pulmonary hypertension often denies pathological sampling as a definitive diagnostic tool. This diagnostic dilemma can be fatal to those patients with PCH treated for pulmonary hypertension because a fatal pulmonary edema may occur. We report a case of PCH in a 26-year-old patient who died of severe circulatory compromise while undergoing transplant evaluation. A discussion of the computed tomography findings, a review of the pathological features, and a review of the relatively limited imaging literature available on this subject are conducted.