Osteogenesis imperfecta is an inherited disorder of connective tissue caused by type I collagen defects, thus all tissues rich in type I collagen are affected. The present report describes a 10-year old Saudi female child with osteogenesis imperfecta and dental problems. Oral and para-oral examination as well as general evaluation was done to the patient. The patient was found to have mandibular osteoporotic changes, delayed eruption of some teeth, caries and malocclusion. Dental treatment was carried out with a follow up of more than 2 years. Based on the previous, patients with osteogenesis imperfecta should be followed up by the dentist collaborated with the treating physician to treat the existing dental problems and to avoid oral and para-oral complications.