Sickle cell disease is a common hereditary hematologic disorder characterized by the presence of a structurally abnormal hemogiobin molecule. Physicochemical properties of sickle hemoglobin result in a chronic hemolytic anemia and in vaso-occlusive episodes with ischemic injury of many tissues. In patients with sickle cell disease the otolaryngologist may be confronted with lesions affecting the ear, the bones and soft tissues, and the nervous system. Surgery and anesthesia also present special problems. An outline and discussion of these clinical concerns are presented.