Adrenal gland involvement as well as metastatic subcutaneous nodule from skeletal osteosarcoma are two extremely rare and unusual manifestations in the natural history of the disease. We herein report a 45 yr old female with both these uncommon occurrences, having large bilateral adrenal metastases and a metastatic subcutaneous nodule in the chest wall along with pulmonary metastasis arising from osteosarcoma of the mandible. Our fluorine -18 fluorodesoxy glucose- positron emission tomography study provided all information needed about the disease status in a single examination. It is noteworthy that osteosarcoma of the jaws, thought to be relatively less aggressive compared to its counterpart in long bones, can occasionally give rise to widespread metastases, including atypical sites. A systematic review of the existing literature aiming to explore the patients’ characteristics and clinical behavior of adrenal metastases from osteosarcoma, including the present case, was carried out. This was nearly always associated with pulmonary metastases with occasional association with brain or skeletal metastases. Peripheral long bones were the overwhelmingly common site of the primary, the present one being the first report of jaw bone being the primary site, giving rise to adrenal metastases. No age predilection was observed with male to female ratio of 3:1 in the small number of reported cases.