Late diagnosed case of testicular feminization with Sertoli Leydig Tumor

Testicular feminization (tf) has a similar phenotype with mayer-rokitansky-kuster-hauser (mrkh) syndrome in patients with amenorrhea, causing confusion in diagnosis. it is an androgen insensitivity syndrome (ais) and is genetically transmitted as an x-linked recessive condition. cytogenetic analysis of patients reveals a 46xy chromosome structure. testicular feminization (tf) has an annual prevalence of 1/20000 and 1/60000, and the main complaint of patients is usually primary amenorrhea. phenotypically, well developed breast tissue, hypertrophic clitoris and labia majora with a short and blind vagina are detected. the aim of this study is to present a tf case that was followed as mrkh syndrome for years, and admitted to our clinic because of a pelvic mass. sertoli leydig cell tumor was diagnosed pathologically during surgery.