The oculocerebrorenal syndrome of Lowe (OCRL) is a rare X-linked recessive disorder, chiefly characterized by ocular involvement, mental retardation, and kidney disease. A literature review is provided, detailing the diversity of oral anomalies associated with the OCRL syndrome. Reported abnormalities include delayed tooth eruption, odontogenic cyst formation, and constricted dental arches. In addition, we present an unusual case of an 18-year-old male affected with the OCRL syndrome and fetal alcohol syndrome. The oral radiographic examination was significant for multiple impacted permanent teeth, many with pericoronal radiolucencies, and an underdeveloped mandible.