Primary renal lymphoma is a rare disease. There is still no agreement on its development primarily in this non-lymphoid organ. Some of the reported cases are questionable because of the incomplete staging of extrarenal disease. In the present study, we describe a rare case of a 73-year-old white man presenting with right flank pain. Physical examination did not reveal any peripheral lymphadenopathy or hepatosplenomegaly. An abdominal computerized tomography (CT) showed a large tumor in the right kidney with no enlarged liver, spleen or lymph nodes. On morphological and immunohistochemical examination, the tumor fulfilled the criteria of anaplastic large cell lymphoma (ALCL). A review of the literature revealed that this is the first reported case of primary ALCL of the kidney.