Some mendelian (monogenic) disorders directly conferring increased susceptibility are associated with diverse infectious organisms, whereas others are restricted in scope to specific genera or even to one species. So far, most investigations of primary immunodeficiency disorders have focused on those conferring susceptibility to viral, bacterial, or mycobacterial infections, providing powerful insight into human determinants of host resistance to these microbes. Monogenic disorders that increase susceptibility to fungal infections are increasingly being recognised. Although infections associated with these disorders are probably less common than are iatrogenic associated mycoses, they provide valuable insight into human immunity to fungal infections. Investigation of these immunological pathways will ultimately lead to improvements in management of such infections in secondarily immunocompromised patients.