Hydrocephalus affects the central nervous system as a result of progressive ventricular dilatation from the accumulation of cerebrospinal fluid in the brain’s lateral ventricles. This paper reports on the oral characteristics of a child with congenital hydrocephalus, discusses her complex dental care needs, and presents dental management of this case. Despite the complex and challenging dental needs, this child received dental treatment in a chairside approach without general anesthesia. A thorough knowledge of the patient’s medical condition, together with expert clinical skills, was indispensable for managing the child and improving the quality and length of her life.