We report a case of pure gonadal dysgenesis with streak gonads in which spontaneous conception occurred 10 years after the diagnosis. Her pregnancy proceeded as normal, and she gave birth to a live baby at term by cesarean section. A lactation period lasting for 1 year and afterwards proceeded as amenorrheic. Gonadotropins measurements in post-lactational period were at the menopausal levels again. To the best of our knowledge, this is the first case of pure gonadal dysgenesis with streak gonads in which spontaneous conception occurred.
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