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Multiple myeloma is a biologically diverse, malignant disease that involve plasma cells. Multiple myeloma is characterized by the unrestrained proliferation of monoclonal plasma cells within the bone marrow. In the present case report, the case of a human immunodeficiency virus (HIV) positive patient with an initial manifestation of extramedullary myeloma involving the parotid gland is described. The patient had been on antiretrovirals for the last two years. A Medline, Embase, Scopus and Google Scholar search was performed using the key