In one case, a mutation in the ABCC8 gene co-occurred with a pathogenic variant in the POMC gene, while, in another case, a pathogenic variant in the KCNJ11 gene was found alongside a mutation in the ADCY3 gene, which encodes adenylate cyclase type 3, a protein that converts ATP to cAMP and is associated with obesity. The gene discussed is POMC; the disease is obesity due to melanocortin 4 receptor deficiency.