Pulmonary capillary hemangiomatosis is a locally aggressive benign vascular neoplasm of the lung characterized by the presence of numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through the pulmonary interstitium, in and around pulmonary vessels and airways. Pulmonary capillary hemangiomatosis is a rare disease characterized by pulmonary hypertension and a slowly progressive clinical course; it is frequently misdiagnosed clinically as primary pulmonary hypertension and veno-occlusive disease. The purpose of this review is to describe the clinical, radiologic, and histologic features of this rare form of pulmonary vascular neoplasm, which may present considerable diagnostic problems to both the clinician and the histopathologist. Fourteen cases of pulmonary capillary hemangiomatosis have been previously reported. In this review we describe the fourth case of pulmonary capillary hemangiomatosis in which the diagnosis was made antemortem, as well as the fourth to undergo lung transplantation.