Rieger's syndrome is a rare autosomal-dominant disorder characterized by dental, ocular, and periumbilical abnormalities. Essential ocular features include goniodysgenesis, which leads to increased intraocular pressure and subsequent glaucoma and visual loss. Dental findings include midfacial hypoplasia and the absence of primary and permanent incisors and second premolars. Comprehensive preventive dental care in the form of fluorides and sealants is required, especially in patients with severe hypodontia, to increase the resistance of the remaining teeth to dental caries. This article describes a case of Rieger's syndrome in a 9-year-old child and emphasizes the need for comprehensive oral rehabilitation.