Introduction:

Disorder of sex development are a sensitive and stressful condition. Many factors influence the decision of sex assignement. Congenital adrenal hyperplasia is the most frequent cause of female disorder of sex development with a challenging management.

Methods:

A retrospective study of 18 patients with female disorder of sex development due to congenital adrenal hyperplasia. Diagnosis and surgical management is reviewed.

Results:

The age ranged from 08 months to 23 years old. Dehydratation was the main feature of diagnosis in 12 patients. Thirteen patients had 21-hydroxylase deficiency. Most of the patients were classified Prader III and IV with respectively 11 and 4 patients. The surgical management was total urogenital mobilization in 13 patients

Conclusion:

The surgical management of congenital adrenal hyperplasia is challenging and controversial