Primary adrenal lymphoma (PAL) is an extremely rare neoplasm of T-cell origin. Here we describe a 42-year-old woman who suffered from intermittent fevers and night sweats of 3 months duration. Laboratory results, imaging, pathology, and immunohistological examination led to a final diagnosis of primary adrenal T-cell lymphoma (PATL) with adrenal insufficiency. The patient received combination chemotherapy and prednisolone but expired 2 months after the treatment. Most reported PAL patients who have received only one therapeutic modality have unsatisfactory survival rates resulting from tumor recurrence and/or postchemotherapy infections. In contrast, one patient who received an adrenectomy followed by low-dose radiotherapy experienced long-term survival. A combination of therapeutic modalities may improve prognosis for patients with PATL, although the case numbers are too small to draw any conclusions.