However, based on a recent revision of gene-disease associations, the list of MODY-causative genes should be restricted to the following: HNF4A, GCK, HNF1A, PDX1, HNF1B, NEUROD1, CEL, INS, ABCC8, KCNJ11, along with RFX6, recently proposed as an additional MODY gene [6]. The gene discussed is HNF1B; the disease is MODY.