Regional odontodysplasia is a rare developmental anomaly of the teeth that affects enamel and dentin. Its etiology is unknown, but the most accepted theory refers to circulatory disorders associated with vascular nevi. An uncommon case of regional odontodysplasia involving three quadrants of the jaws is reported. An 8-year-old boy was referred to the authors' institution with lack of teeth as a major symptom. There was no hereditary anomaly in the family. Extraoral examination revealed a brown discoloration on the skin on the right side of the face, neck, and chest. Detailed intraoral examination showed hypoplastic and carious teeth. Fistulae and gingival overgrowth were also observed. Radiographically, the affected teeth presented the pathognomonic image of "ghost teeth." Conservative treatment was instituted. Keywords: diagnosis