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Myotonic dystrophy 2 (DM2) is a genetic multi-systemic disease primarily affecting skeletal muscle. It is caused by CCTGn expansion in intron 1 of the <i>CNBP</i> gene, which encodes a zinc finger protein. DM2 disease has been successfully modeled in <i>Drosophila melanogaster,</i> allowing the identification and validation of new pathogenic mechanisms and potential therapeutic strategies. Here, we describe the principal tools used in <i>Drosophila</i> to study and dissect molecular pathways related to muscular dystrophies and summarize the main findings in DM2 pathogenesis based on DM2 <i>Drosophila</i> models. We also illustrate how <i>Drosophila</i> may be successfully used to generate a tractable animal model to identify novel genes able to affect and/or modify the pathogenic pathway and to discover new potential drugs.