Osler-Weber-Rendu syndrome is characterized by widespread telangiectasias. Its clinical manifestations depend on position and scope of the abnormal vessels. The clinical and CT data of 3 patients with Osler-Weber-Rendu syndrome were retrospectively analyzed. CT features reviewed include the change of volume and configuration of liver, presence of tortuous and irregular vessels, opacified vessel mass, arteriovenous shunt, and splenomegaly. CT is helpful for diagnosis, treatment, and follow-up of Osler-Weber-Rendu syndrome.