Primary adrenal lymphoma is extremely rare. We describe a case of non-Hodgkin's lymphoma of diffuse large B-cell type with right adrenal involvement. The patient received chemotherapy and external irradiation and achieved complete remission of the disease. We describe the case of primary adrenal lymphoma with a review of the literature on this unusual neoplasm. Primary adrenal lymphoma should be included in the differential diagnosis of adrenal mass.