To get better understanding of the disease, we analyzed the clinical characteristics and gene mutations of MODY5.<h4>Methods</h4>PubMed, Cochrane, the China National Knowledge Infrastructure, and Wanfang were searched with the following search terms: "MODY5" OR "HNF1B maturity-onset diabetes of the young" OR "maturity-onset diabetes of the young type 5" OR "renal cysts and diabetes syndrome". Here, HNF1B is linked to type 2 diabetes mellitus.