To date little data exist about treatment of hematologic malignancies in patients with end-stage renal disease (ESRD). While administration of immunochemotherapy comprising the CD20-antibody rituximab is a well-established treatment strategy in patients with normal renal function, little information on safety and efficacy is available in the setting of ESRD. Here we describe for the first time a hemodialysis patient suffering from diffuse large B-cell Non-Hodgkin's lymphoma (DLBCL) who was treated with polychemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) in combination with rituximab (R-CHOP). We observed no major adverse events and treatment resulted in a partial remission of the DLBCL. Thus, administration of R-CHOP may be considered as a safe therapeutic option in this setting. Of note, this patient had a previous history of hairy cell leukemia. A review of the literature was performed and the potential etiologic link of these two B-cell malignancies is discussed in the light of available information.