Caspase recruitment domain-containing protein 9 (CARD9), one of the CARD family members, is an essential adapter in myeloid cells, and is expressed in various tissues, including the spleen, liver, placenta, lung and brain. By forming a complex with Bcl-10 and mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1), CARD9 mediates C-type lectin receptors (CLRs)-triggered intracellular signaling and nuclear factor κB (NF-κB) activation, which is therefore crucial in antifungal immunity. Evidence indicates that CARD9 mutations lead to increased susceptibility to various fungal infections. To date, 18 missense and nonsense CARD9 mutations have been reported in 56 patients from 14 countries, which is linked with a variety of fungal infections including Candida, Dermatophytes, Dematiaceous fungi, Aspergillus, etc. This review aims to summarize recent progress in CARD9 mutation and different fungal infections.