Abstract
Disorders of sex development (DSD) commonly result from testicular dysfunction either due to developmental defect resulting in complete gonadal dysgenesis (CGD) or androgen synthesis/action causing congenital adrenal hyperplasia (CAH) or androgen insensitivity syndrome (AIS) and are phenotypically females with diverse clinical presentation. CGD is typically associated with absent breast development, streak gonads and hypoplastic mullerian structures. We report two cases of CGD with non-classical presentations highlighting the heterogeneous presentation this diagnosis can have.
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