Conclusion
This rare case highlights the co-existence of CAH and Jacob syndrome (47, XYY Syndrome) that leads to ambiguous genitalia and various urogenital abnormalities and underscores a stepwise diagnostic approach in patients with atypical presentation. A multidisciplinary approach including endocrinology, surgery, and genetics is necessary for the proper management of complexities associated with the condition. Further research is needed to find the association between CAH and Jacob syndrome in patients with ambiguous genitalia.