Differential diagnosis of generalised rhizomicry (root dwarfism) includes many diseases and syndromes. When the patient has normal stature with no evidence of ghost teeth, no systematic diseases and no history of irradiation or chemotherapy, the dental features are pathognomonic for the diagnosis of dentine dysplasia type 1 (DD-1). In this report, we presented an adult case with DD-1 that had been left undiagnosed and the patient underwent dental implant therapy and orthodontic treatment, despite recognition of early tooth loss. The diagnosis of DD-1 was first established before an orthognathic surgery to correct the facial skeletal deformity. We also reviewed the clinicopathological aspects of this disease and clinical dental implications for this patient group.
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