Marked pulmonary hypertension developed in a 40-year-old man with known cirrhosis and a previous portosystemic shunt. Terminally, he also showed signs of microangiopathic hemolytic anemia. At postmortem examination, he had severe plexiform dilatation lesions in the pulmonary vasculature, with deposition of fibrin in the vasculature channels. It is suggested that the site of microangiopathic red cell damage was the pulmonary microvasculature.