[Male XX-syndrome].

Described in this paper is a patient, aged eleven years and eight months, with micropenis, dorsal prepuce, and rugous, partially bifid scrotum. FSH and LH values below normal were obtained from hormonal investigation. LH-RH stimulation was followed by retarded FSH response of moderate intensity, while the LH profile was moderately strong. Cytogenetic tests revealed the presence of 25 per cent of female sex chromatin. The karyotype was 46 XX. The patient did not have internal female genital organs, and his gonads were of testicular appearance. Histologically, both testicles exhibited tubules with primitive germinal cells and Sertoli cells. An inconspicuous number of Leydig cells was recorded from the interstitium.