MODY is a genetically heterogeneous disease and to date 14 genes (GCK, HNF1A,HNF4A, HNF1B, INS, KCNJ11, ABCC8, PDX1, NEUROD1, KLF11, CEL, PAX4, BLKand APPL1) were associated with MODY. The gene discussed is ABCC8; the disease is MODY.