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We report a rare case of osteoglophonic dysplasia affecting father and daughter. Osteoglophonic dysplasia is a very rare skeletal dysplasia with craniosynostosis, multiple radiolucencies of bone and clinical anodontia. It is an autosomal dominant disorder characterised by short stature. The affected children have normal intelligence. Close association with missense mutation of fibroblast growth factor receptor-1 has been reported. Life expectancy depends on the degree of cranial malformation. In previous reports, bone defects usually resolved by adulthood, but multiple tooth impaction may cause aesthetic and masticatory problems. Cytogenetic studies and routine laboratory tests were all within normal limits.

Neurilemmoma is a benign nerve sheath tumour that is composed entirely of well-differentiated Schwann cells. Schwannomas arise when proliferating Schwann cells in the perineurium causing displacement and compression of peripheral, motor, sensory, sympathetic and cranial nerve sheath. They may occur at any age, but the peak incidence is 3rd and 4th decades with no sex predilection. Histopathological picture shows primarily of Schwann cells in a poorly collagenized stroma. The treatment of choice is surgical excision with good prognosis and rare secondary complications. We describe a clinical case of intraosseous schwannoma located in the right pretragus area in a 48-year-old female patient. It is important to highlight that the schwannoma is usually found in the head and neck, and rarely in the oral cavity. Intraosseous neurilemmomas are rare, and they represent less than 1% of all benign primary bone tumours.

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