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Infection is an important cause of morbidity and mortality among patients with end stage renal disease. Stenotrophomonas maltophilia is an unusual yet emerging pathogen in dialysis units. We performed a systematic PubMed/Medline and Scopus review of peer-reviewed English papers on S. maltophilia infections among patients undergoing chronic dialysis, with regard to vascular accesses, systemic infections and environment contaminations. Moreover, we suggest a treatment algorithm to preserve the patient and the permanent dialysis catheters.