Regional odontodysplasia (ROD) is a rare dental anomaly that can affect deciduous and permanent dentition. This unique dental abnormality involves enamel, dentin, pulp, and dental follicle. ROD has a prevalence of less than 1:1.000.000 and the age at the time of diagnosis ranges from 1 to 23 years. Females are slightly more affected than males at a ratio of 1.4:1. The maxilla is affected twice as often as the mandible. The aetiology of ROD is still unknown and conditions such as viral infections, local trauma, vascular defects, irradiation, metabolic disturbance, rhesus incompatibility and medications during pregnancy have been suggested as possible causes. The diagnosis is usually made by clinical and radiographic findings. Generally, the affected teeth fail to erupt, but when erupted, they are small, hypoplastic, or hypocalcified with deep pits and clefts which could be connecting the pulp. Radiographically, the affected teeth show a typical "ghost-like" appearance. The affected teeth have large pulp chambers and short roots with open apices. The treatment of ROD is controversial. It is widely accepted that implant-supported single-unit crowns represent a reliable treatment option for the replacement of missing teeth with favourable outcomes. The objective of this review and reported case highlights epidemiology, etiopathogenesis, diagnosis, and implant treatment modality.