Based on the known function of FA2H and its role in the skin, the predicted impact of the identified variant and its perfect co-segregation with the disease phenotype in the studied pedigree, we conclude that inherited IC in Chianina cattle is caused by a homozygous loss-of-function variant in FA2H. Thereby, this study represents an outstanding animal model for the understanding of similar conditions in different species and adds FA2H to the list of candidate genes for ichthyosis in humans. The gene discussed is FA2H; the disease is ichthyosis.