To summarize, the susceptibility to endemic mycoses in CD40L deficiency, IL12Rβ1 deficiency, and IFN-γR1 deficiency highlights the critical role of the IL-12/IFN-γ crosstalk in macrophage activation and killing of these endemic fungi, while the deficiency of Th17 cells in patients with GOF STAT1 defect and AD hyper-IgE syndrome puts them at risk for both CMC and IFIs, and they frequently have CMC due to impaired mucosal immunity against C. albicans. This evidence concerns the gene IFNGR1 and hyperinsulinemic hypoglycemia, familial, 4.