Giant cell rich osteosarcomas, histological variant of conventional osteosarcomas account for 3% of all cases of osteosarcomas and most of them are arise from the appendicular skeleton. Giant cell rich osteosarcomas have been rarely reported in the head and neck region. This is the first case report of giant cell rich variant of osteosarcoma in the maxilla. It is important to recognize this variant, as its prognosis is worse as compared to conventional osteosarcomas of the head and neck region. We here present a case of giant cell rich variant of osteosarcoma of the maxilla and along with its characteristic radiological and histopathological picture.

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Purpose The authors intend to present a case of osteosarcoma of the maxilla which presented like a mucocele, a previously unreported phenomenon. Patients and methods Clinical features, radiology, histopathological findings and endoscopically managed osteosarcoma of the maxilla are presented. Diagnostic difficulties and previously reported diagnostic dilemmas related to osteosarcomas of the maxillary sinus are discussed. Results The patient has been on regular follow-up for the past 10 months, and is free from any local or systemic disease. Conclusion Though osteosarcoma of the maxilla is a rare tumor about 6.5% of all osteosarcomas1, patients tend to be in the late-third to mid-fourth decades of life, a high index of suspicion and aggressive management can reduce the morbidity that goes with the management of this condition.