Presence of bilateral fallopian tubes with measurable müllerian-inhibiting substance in a true hermaphrodite

Abstract A case of true hermaphroditism is presented in which the serum mullerian-inhibiting substance (MIS) level was discordant with the phenotype predicted according to the Jost principle. The implication of this finding with respect to both the pathophysiology of true hermaphroditism and the role of MIS in the management of intersex conditions is discussed.