Myotonic dystrophy type1(MD1) represents an autosomal dominant genetic disorder with the properties of multisystem getting involved. The organ systems implicated are skeletal muscle, heart, gastrointestinal tract( GIT), as well as brain There have been reports regarding the implications of changes in insulin signaling as well as their association with the clinical MD1 features. Thus we decided to conduct a systematic review regarding this. We utilized the PubMed search engine, Web of Science, Google Scholar, Embase, and Cochrane reviews using the MeSH terms like Myotonic dystrophy type1 & type2; insulin signaling; insulin receptors(IR-A&B); control of insulin signaling in the brain other disorders related to enhancing age comparison; insulin-like growth factor1IGFR 1receptor); comparison with alterations in diabetes type 2 (T2DM) as well as metabolic syndrome(MetS) from 1970 to date in 2020 on 31st August 2020. We found over 10,150 articles out of which we selected 112 articles for this review, No meta-analysis was done. It was seen that the clinical proof of implications of the insulin signaling pathway in MD1 is dependent regarding enhanced chances of IR observed during practice as well as current research proofs regarding the benefits of metformin with regards to muscular action. Further indirect proof comes via some central nervous system(CNS) obtained symptoms that are typical of MD1, like obsessive compulsive behaviours or obsessive-compulsive disorders( OCD), whose association with changes in insulin signaling was shown in different disorders. On ground system, various etiopathological along with physiological modes which are working in MD1 might interfere with the usual physiological action of insulin. With all this proof the significance of insulin signaling in association with clinical features of MD1 warrants more basic scientific as well as clinical along with therapeutically directed research.