While most reported NEUROD1 mutations cluster within the transactivation domain and are typically associated with classic MODY6 phenotypes—characterized by non-ketotic, insulin-independent diabetes (4)—emerging evidence indicates that variants in the 5′ untranslated region (5′UTR) may disrupt transcriptional regulation, potentially resulting in atypical clinical presentations, including diabetic ketosis (8, 9). The gene discussed is NEUROD1; the disease is maturity-onset diabetes of the young type 6.