Over the past decade, awareness of the association between portal hypertension and changes in the intestinal circulation has increased. Most of the observations have been made by endoscopic examination and biopsy of the mucosa. The fundamental pathologic change is a vasculopathy. Portal hypertensive intestinal vasculopathy (PHIV) most often involves the stomach (gastropathy) and can be a common source of bleeding. The significance of small bowel involvement (enteropathy) is unknown. Colon involvement (colopathy) has been associated with bleeding, and mimics inflammatory bowel disease. The reliability of endoscopic appearances and histologic examination in establishing the diagnosis is questionable. Recent observations of other diagnostic modalities and associated physiologic alterations and treatment options are discussed. Further prospective evaluations that use uniform terminology for endoscopic and histologic descriptions are needed to establish criteria for accurate diagnosis and assessment of response to treatment.