Persistent Mullerian duct syndrome with bilateral total absence of Wolffian duct structures

Persistent Mullerian duct syndrome was found in a phenotypically normal male with a normal 46XY karyotype. Bilateral total absence of Wolffian duct structures was encountered during surgery. Although many cases with one or a combination of abnormalities of Wolffian duct structures have been described, to our knowledge, this finding has not been previously reported. Clinical, hormonal and radiological findings are presented, possible explanations are discussed