A patient with bilateral primary adrenal lymphoma, presenting with fever of unknown origin and achieving long-term disease-free survival after resection and chemotherapy.

Primary adrenal lymphoma is extremely rare. We describe a 64-year-old female patient who presented with fever of unknown origin. Imaging studies demonstrated bilateral bulky adrenal masses. She underwent bilateral adrenalectomy and the pathological diagnosis was large cell immunoblastic (B-cell) lymphoma. She received adjuvant combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone in the following 6 months. She has been relapse free for 52 months. To the best of our knowledge, this case has the longest disease-free survival among those reported. The present case indicated that primary adrenal lymphoma should be included in the differential diagnosis of fever of unknown origin and/or suprarenal mass. Chemotherapy following surgical resection may be considered the treatment of choice.