Few data are available about sorafenib use in patients with metastatic renal cell carcinoma (mRCC) undergoing hemodialysis. No systematic review has been previously performed about this issue. The objective of the present review is to investigate pharmacokinetics and clinical outcomes of sorafenib in mRCC patients undergoing hemodialysis. According to PRISMA guidelines, all the literature about mRCC dialysis patients receiving sorafenib, published from January 1946 to August 2015, was evaluated. Applying inclusion/exclusion criteria, 11 articles were selected for the analysis; one patient from our department was also included. The investigated outcomes were pharmacokinetics, toxicity, response rate (RR), progression-free survival (PFS) and overall survival (OS) where available. A total of 36 patients were included. Median treatment duration was 6.0 months on overall population; median PFS was 6.3 months (calculated on 19 patients); RR was 22% (on 29 patients); median OS was 14.9 months (on 28 patients). Of note, 24 patients started Sorafenib at reduced dose; 6/36 patients (17%) required dose reduction due to adverse events (AEs). Sorafenib treatment was discontinued in seven patients (19%) because of AEs. Most of AEs were Grade 1-2; severe toxicities (Grade 4-5) included G4 anemia (one case), G4 hypertension (one case), G4 cerebellar haemorrhage (one patient) and a case of G5 subarachnoid haemorrhage. This review confirmed the efficacy of sorafenib treatment in mRCC patients receiving hemodialysis. Nevertheless, drug toxicity seems to be increased in these patients, despite the initiation of therapy at reduced doses, therefore sorafenib should be used with caution in dialysis patients.