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Primary adrenal lymphoma (PAL) is a sporadic malignant disease characterized by a high level of invasiveness and a poor prognosis. Symptoms are atypical, making it difficult to obtain an accurate early diagnosis. Some patients may have fever, night sweats, weight loss, lumbar and abdominal pain, and adrenal insufficiency. Certain clinical signs and biochemical characteristics can help clinicians make an early and rapid diagnosis to prevent a delay in treatment. Here, we report a case of a female patient with unilateral PAL and low back pain. After rapid diagnosis, R-CHOP chemotherapy combined with autologous peripheral stem cell transplantation was administered; the patient's condition improved significantly, and her prognosis was good. There was no recurrence during a follow-up period of more than two years.