The Osler-Rendu-Weber syndrome is characterized by multiple telangiectasic lesions usually involving the mucous membranes, face and distal extremities. It is a congenital malformation inherited as an autosomal dominant trait and the lesions usually appear during adulthood. The major symptoms are recurrent epistaxis and gastrointestinal bleeding, but they may cause intracranial hemorrhage at the white matter of the brain stem, cerebellum and diencephalon. We report a case of typical autosomal dominant trait Osler-Rendu-Weber syndrome associated with intracranial hemorrhage at the right basal ganglia.