Hydrocephalus is considered a congenital disease affecting the central nervous system, and is characterized by accumulation of cerebrospinal fluid in the lateral ventricles of the brain causing progressive ventricular dilatation. This review paper reports on the oral characteristics of persons with hydrocephalus and discusses their dental care needs. It has been reported that patients with hydrocephalus can present chronological changes in the eruption of their teeth, changes in the occlusion, greater accumulation of plaque, and have a higher caries prevalence. Regular dental visits are needed to control caries as well as to educate the caretakers about oral hygiene for children with hydrocephalus. In addition, the dental practitioner should be careful not to put pressure on the ventricular-peritoneal drainage tube during dental treatment. Special devices are available to support the patient's head if they have macrocephally.