Rendu-Osler disease is a rare, autosomal dominant vascular malformation disorder with diverse clinical manifestations. It commonly presents with recurrent epistaxis, iron deficiency, and secondary anemia. The condition affects small vessels in the nasal, oral, and gastrointestinal mucosa, the skin of the face, lips, and fingertips, and solid organs. This article discusses general and specific manifestations of the disease, along with its general and particular management. A case presentation is included to demonstrate that treatment must be individualized, taking into account all aspects of the condition, including local and systemic manifestations, comorbidities, and the patient’s chronic treatment.