| Summary: | <b>Background: </b> Ambiguous genitalia are a major cause of parental anxiety and can create social problems if not properly managed. Diagnosis and management can however be challenging. The aim of this study is to highlight some of the challenges in management of ambiguous genitalia in our environment. <b> Patients and Methods:</b> All cases of ambiguous genitalia managed at the Paediatric surgical unit of the Obafemi Awolowo University Teaching hospital, Ile Ife, Nigeria, between January 1993 and October 2007 were analysed for age, sex at presentation, investigation modality, and final sex of rearing and outcome of surgery. <b> Result: </b> Nine patients had surgical reconstruction for ambiguous genitalia during the study period. Their age ranges from 5 weeks to 19 years at presentation. The causes of genital ambiguity in the patients was congenital adrenal hyperplasia (CAH) in 6, true hermaphroditism in 2 and male pseudo-hermaphroditism in 1. Seven patients were reconstructed as females while 2 were raised as males. Change of sex of raring was necessary in 2 patients. <b> Conclusion: </b> The diagnosis and management of ambiguous genitalia is a challenging problem in our environment. Early presentation and treatment is necessary to avoid psychological and social embarrassment.
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