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Abstract

Disorders of sex development (DSD) are congenital abnormalities of sexual differentiation resulting from disparity in the chromosomal, gonadal and phenotypic sex. Most of these children present with ambiguous genitalia, but can also present with hernias or undescended testis as in persistent Mullerian duct syndrome (PMDS). The social stigma attached with this disorder makes it imperative for expeditious evaluation and appropriate assignment of sex in our scenario. We report here successful management of such a case and review the literature. In conclusion, a rare case of DSD like PMDS requires a high index of suspicion and can be managed successfully.

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58

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60

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Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License
This work is licensed under a Creative Commons Attribution-NonCommercial-Share Alike 4.0 International License.

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