Plasmablastic lymphoma is a high-grade B-cell lymphoma that more commonly affects the oral cavity of immunosuppressed patients. EBV is present in approximately 70% of the cases and is believed to play an important role in the development of this neoplasm. Chromosomal translocation involving the MYC gene is found in 50–60% of the cases. Adult males are the most affected, usually in their fourth to sixth decades of life. The gastrointestinal tract and the skin are other locations frequently affected. The neoplasm usually presents as rapidly growing tumors, with ulcerated regions and areas of necrosis. The mucosa of the upper gingiva/alveolar ridge and the palate is the most affected location in the oral cavity. Microscopically, there is a diffuse infiltration of tumor cells, with the presence of tingible body macrophages that give rise to a “starry sky” pattern, predominance of large neoplastic cells (plasmablasts) that express plasma cell markers and lack CD20 expression. There is currently no gold standard chemotherapeutic approach for treating the affected patients, and different regimens have been applied. Bone marrow transplant and adjuvant radiotherapy have also been used. Overall prognosis is poor and survival achieves a median of 6–7 months.