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Summary  We identified an adolescent young woman with new-onset diabetes. Due to suspicious family history, she underwent genetic testing for common monogenic diabetes (MODY) genes. We discovered that she and her father carry a novel variant of uncertain significance in the  HNF1A  gene. She was successfully transitioned from insulin to a sulfonylurea with excellent glycemic control. Based on her family history and successful response to sulfonylurea, we propose that this is a novel pathogenic variant in  HNF1A . This case highlights the utility of genetic testing for MODY, which has the potential to help affected patients control their diabetes without insulin.    Learning points     HNF1A  mutations are a common cause of monogenic diabetes in patients presenting with early-onset diabetes and significant family history.    Genetic testing in suspected patients allows for the identification of mutations causing monogenic diabetes.    First-degree relatives of the affected individual should be considered for genetic testing.    The use of sulfonylurea agents in patients with HNF1A-MODY can reduce dependence on insulin therapy and provide successful glycemic control.

Identification of a novel hepatocyte nuclear factor-1 alpha (HNF1A) variant in maturity onset diabetes of the young type 3 (HNF1A-MODY)