Osteogenic imperfecta is one of the most common inherited bone diseases. The disease typically involves the bones, teeth, ligaments, eyes and skin and is characterized by bone fragility. Aim: To estimate the prevalence and the clinical characteristics of osteogenic imperfecta in the Dakhliya region of the Sultanate of Oman. Method: Retrospective study of all cases of OI diagnosed and followed in Nizwa hospital since 2000. Data was collected from the case files obtained from the medical record department. Result: A total of 22 patients were identified in the study 10 (45.5%) were male and 12 (54.5%) were female. The mean age of diagnosis was 6.46 years. The parents of all patients had a first degree consanguineous marriage. A total of 66 fractures were sustained in these patients. Conclusion: Osteogenic imperfecta is an important disease in Oman with high morbidity and mortality. There are new modalities of treatment like Bisphosphante which have been studied recently and showed remarkable improvements in the bone mineral densities of these patients. We suggest starting an OI registry in the country. This will enable the scientist and the medical professional to diagnose, treat and study these patients appropriately and promptly.