Myeloid sarcoma (MS) is a rare extramedullary tumor composed of primitive granulocytic cells. These lesions are commonly associated with other hematologic disorders such as myeloid leukemia and other myeloproliferative neoplasms. Although extremely rare in the oral cavity, this lesion was reported in gingiva, palate, buccal mucosa and extraction sockets. MS is an aggressive lesion associated with poor prognosis. Early identification and prompt treatment holds the key for increasing the disease-free period in these patients. In this context, we report a rare and aggressive case of MS, which ran a fatal course in a 45-year-old female patient.