Aim: The purpose of this article is to report some unusual characteristics related to gender, location and manifestations of severe Regional Odontodysplasia detected in three subjects. Regional Odontodysplasia is a rare disturbance of dental development whose aetiology is still unknown. Anomalies involve enamel, dentin, pulp and dental follicle causing atypical structure, colour, shape, size and eruptive disturbances of the affected teeth. Its early onset may lead to craniofacial development disturbances.
Cases reports: This article reports three cases with unusual characteristics observed in male children who were assisted by a paediatric dentist. Generally the disease affects one hemiarch and it is very rare that it crosses the midline as in one of the presented cases. Regional Odontodysplasia has been predominantly described in the maxilla and in women, however these three cases are in boys and two of them occured in the mandible.
Conclusion: Regional Odontodysplasia is a rare disease, causing severe dental, growing and craniofacial development anomalies. Treatment needs to be personalised, aiming at preservation of the affected teeth taking into account their risk to develop severe infections. Parents should be made aware of the need for an extensive follow-up.