Hereditary hemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber (ORW) disease is an autosomal-dominant disorder that frequently presents with gastrointestinal bleeding which may be a diagnostic and therapeutic challenge. Hepatic involvement in this disease is increasingly recognized and poses another therapeutic challenge. With advances in genetic screening and diagnostic procedures, and the increasing awareness of the condition by physicians and patients, this disease is being diagnosed more often. This article reviews the available literature on gastrointestinal and hepatic manifestations of HHT, and the various diagnostic and therapeutic modalities available for its management.