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Regional Odontodysplasia is a rare developmental anomaly with unknown cause affecting ectodermal and mesodermal dental layers, affecting segment of jaw more commonly maxilla. A case of regional Odo-todysplasia was reported in a 13 year old patient in whom maxillary right segment was affected. Teeth affected were central incisor, canine, first & second premolar with different grades of severity & lateral incisor appeared to be nonnal. Affected teeth were having irregular, rough & discolored surfaces, localized gingival enlargement, periapical pathosis, radiographically "ghost. tooth" appearance &. short roots. Additional features noted were impacted permanent canine & overretained deciduous canine in the affected segment.