Sex steroid signalling determines female and male sexual development and maintains the female and male phenotype in adults. Steroids carry out their function by activation of their cognate intracellular receptor, which is a ligand-dependent transcription factor. Steroid receptors function by binding to specific structural elements in the regulatory regions of target genes and by recruitment of cofactors by protein-protein interaction. Cofactors might display enzymatic activities that modify histones and other proteins. Cofactors also include proteins that modulate the chromatin structure and protein complexes that function as bridging factors between the multi-protein complexes. This review focuses mainly on the function of the androgen receptor and its cofactors and their role in androgen insensitivity syndrome.