Severe fungal infections in healthy patients have recently been reported from a few countries, i.e., Algeria, Brazil, France, China, Iran, Morocco and Tunisia (Glocker et al., 2009; Drewniak et al., 2013; Lanternier et al., 2013; Wang et al., 2014; Grumach et al., 2015) and linked to autosomal recessive CARD9 deficiency. Here, CARD9 is linked to fungal infectious disease.