In this study we present the profile of 33 cases with 46, XY DSD. The aim of this work was the proper diagnosis and classification of these cases. The age of the studied cases ranged from 8 days to 29 years. +ve consanguinity was noticed in (84.8%), clinical, hormonal, radiological and sometimes molecular study were done for the patients. The results showed that 15.1% of the patients had disorders of testicular development , 48.4% had disorder in Androgen action and 33.3% had disorders in Androgen synthesis.