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Steroid resistance syndrome (mineralocorticoids, glucocorticoids, estrogens, androgens) is a rare clinical disorder, androgen insensitivity syndrome being the most commonly described. Resistance syndromes are characterized by elevated steroid hormone levels, secondary to an impaired signal transduction and a lack of negative feedback, without any specific clinical signs of steroid excess. In most cases, steroid hormone resistance is generally caused by steroid receptor mutations. Several nonsense and missense mutations or deletions have already been described for all steroid receptors, except for the progesterone receptor. The number of reported mutations is extremely variable, depending on the steroid receptor, ranging from 3 for the estrogen receptor to more than 500 for the androgen receptor. Loss of function of others factors implicated on the steroid hormone signaling pathway, such as molecular chaperones or coactivators, might also be involved in the pathogenesis of steroid resistance. Therapy is essentially symptomatic, there is currently no pharmacological strategy enabling restoration of the impaired hormone signaling pathway. Identification of steroid resistance syndrome and their characterization should allow a better understanding of underlying molecular defects, opening new avenues for the development of novel alternative options for the disease management.