Primary adrenal lymphoma is extremely rare. Here we describe a 32-year-old man who suffered from intermittent fevers and weight loss of 6 months duration. Computed tomography scan found enlarged adrenal glands retaining their characteristic shape with a round homogeneous mass on the right. Laboratory results, imaging, pathology, and immunohistologic examination led to a final diagnosis of primary adrenal T-cell lymphoma. The patient received retroperitoneal laparoscopic adrenalectomy followed by chemotherapy and prednisolone replacement therapy. The patient has survived 3 years.