Dermatophytosis is a widespread disease with high prevalence and a substantial economic burden associated with costs of treatment. The pattern of this infectious disease covers a wide spectrum from exposed individuals without symptoms to those with acutely inflammatory or non-inflammatory, chronic to invasive, and life-threatening symptoms. Moreover, the prevalence of cutaneous fungal infections is not as high as might be expected. This curious disparity in the dermatophyte infection patterns may suggest that there are individual factors that predispose to infection, with genetics as an increasingly well-known determinant. In this review, we describe recent findings about the genetic predisposition to dermatophyte infections, with focus on inheritance in families with a high frequency of dermatophyte infections and specific host–pathogen interactions. The results of studies indicating a hereditary predisposition to dermatophytoses have been challenged by many skeptics suggesting that the varied degree of pathogenicity and the ecological diversity of this group of fungi are more important in increasing sensitivity. Nonetheless, a retrospective analysis of the hereditary propensity to dermatophytoses revealed at least several proven genetic relationships such as races, CARD9 deficiency, HLA-DR4 and HLA-DR8 type and responsible genes encoding interleukin-22, β-defensin 2 and 4 as well as genetic defects in dectin-1, which increased the prevalence of the disease in families and were involved in the inheritance of the proneness in their members. In future, the Human Genome Diversity Project can contribute to elucidation of the genetic predisposition to dermatophytoses and provide more information.