PURE GONADAL DYSGENESIS: CLINICAL CASE AND REVIEW OF THE LITERATURE

XX or XY pure gonadal dysgenesis (PGD) is defined by premature destruction of the fetal gonads which reduce to an undifferentiated stroma with absence of germ line and endocrine secretion.  The phenotype is unambiguously female with a clinical picture associating primary amenorrhea and impuberty. We present in this article a clinical case illustrating this pathology in a young patient who presents a primary amenorrhea. The diagnosis of pure XX gonadal dysgenesis was retained based on the clinical and para-clinical examinations. The patient was treated with hormone replacement therapy. The chances of fertility are almost absent in our context, and the only way out is egg donation.