Lymphomas of the genitourinary tract represent rare tumours for which the diagnosis is crucial regarding the specificity of the treatment. The most frequent localisations are the kidney (solitary tumour or multiple nodules) and the testis; other sites of the genitourinary tract are uncommon. One of the main challenges is to obtain an appropriate immunohistochemical diagnosis with a good staging which is necessary to adapt the therapeutic options. These are mostly based on chemotherapy (with immunotherapy in B-cell lymphomas), of which the intensity and number of cycles depend on initial prognosis factors.