Primary adrenal lymphoma is uncommon, and the majority cases of this disorder are found in elderly individuals. We describe a 17-year-old boy with persistent fever, hemophagocytic lymphohistiocytosis, and a bilateral tumor of the adrenal glands. The disease was progressive and did not respond to treatment such as immunosuppression therapy or plasma exchange. Postmortem analysis revealed nasal-type natural killer cell lymphoma in association with Epstein-Barr virus infection. To our knowledge, this case is the first of primary adrenal lymphoma with the natural killer cell phenotype to be reported. The characterization of this unusual case should be included in the differential diagnosis of adrenal gland tumors.