Primary adrenal lymphoma (PAL) is an exceedingly rare disease. Because of its rareness, uncertain etiology, variable duration of survival, unremarkable clinical presentations and unsatisfied therapeutic strategies, its treatment has always been unsatisfactory. Here we report a 50-year-old male patient with primary bilateral adrenal diffuse large B-cell lymphoma. He was treated with surgery followed by combined chemotherapy using R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). He achieved complete remission after 3 cycles of chemotherapy. At present, he is considered free of tumor at 3 years after chemotherapy. Our case report demonstrates that patients with primary bilateral adrenal diffuse large B-cell lymphoma may achieve a good outcome using R-CHOP chemotherapy. To the best of our knowledge, our patient who achieved complete remission after R-CHOP chemotherapy has had the longest survival as published in the literature.