Background: The aim of the study was to investigate the serum levels of Anti-Müllerian hormone (AMH) in children with disorders of sex development and its correlation with other diagnostic investigations to assess the ability of AMH to predict the presence and function of testicular tissue. Methods: The study was carried out at the pediatric, pediatric surgery and clinical pathology departments at Zagazig University Hospitals (ZUH) during the period from February 2018 to December 2018. The study was performed on 40 infants and children below the age of ten years. They were divided into two groups, control and DSD groups, each group contained 20 participants. Children were subjected to full history taking, precise clinical examination, and investigations that included ultrasonography, karyotyping and biopsies. In addition, serum AMH, testosterone and dihydrotestosterone (DHT). Statistical analysis using suitable significance tests was performed. Results: Specificity and sensitivity of testosterone and DHT in detection of testicular activity were 100% for both while they were 91.7% and 100% respectively for AMH in 46,XY DSD. Conclusion: Serum testosterone and DHT had a better diagnostic role in 46.XY DSD than AMH while in 46,XX DSD, AMH is better.