Abstract: The clinical presentation of primary adrenal lymphoma(PAL) is comprised of major general symptoms. Although adrenal insufficiency is very common in patients with bilateral involvement, it has not been systematically tested. Positron emission tomography is an efficient examination to visualize extra-adrenal locations. The preliminary results of magnetic resonance imaging to distinguish between PAL and adrenocortical carcinoma should be confirmed. Further studies are needed to establish an optimal strategy for the management of patients with PAL. At present, it is unclear whether the adjunction of rituximab (R) and CHOP(cyclophosphamide, doxorubicin, vincristine, prednisone) can cure patients with high-risk aggressive lymphoma. It is also unknown whether the prolonged complete response was due to R-CHOP alone. It is necessary to emphasize that early diagnosis of PAL before the appearance of adrenal insufficiency contributes to decreasing the patients' morbidity and mortality.