Hajdu-Cheney syndrome is a very rare, inherited, autosomal dominant, skeletal dysplasia associated with characteristic craniofacial and dental features, primary acroosteolysis of the terminal phalanges and generalized osteoporosis. A 37-year-old male patient presented with features of osteomyelitis of the right mandible and typical features of Hajdu-Cheney syndrome. The patient also had calcification of the falx cerebri and an unusual median palatal groove, which has not been reported in Hajdu-Cheney syndrome before. The clinical and radiological features, differential diagnosis, and management of the patient are presented.
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