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Primary adrenal Non-Hodgkin′s lymphoma is rare. The symptoms of the disease and response to treatment are variable depending on the type of lymphoma, tumor size, and presence of adrenal insufficiency. We report two cases of primary adrenal lymphoma who had varied presentations. One presenting with abdominal pain and weight loss was documented to have unilateral disease without any adrenal insufficiency and showed a good response to combination chemotherapy, while the second one had bilateral adrenal involvement with adrenal insufficiency and died after second chemotherapy. Functional adrenal involvement in lymphoma depends on the extent of involvement; patients with bilateral involvement almost always have adrenal insufficiency.