Disorders of sex development (DSDs) cover congenital or developmental abnormality of any part of the female or male reproductive tract. In this chapter, the embryogenesis of the genital system and the most common DSD are presented. Knowledge of embryogenesis of the genital tract is very important, because if there are inherited gene mutations, de novo mutations, external factors, or both genetic and environmental factors that interfere with the stages of development, clinically, sooner or later, changes in reproductive function will be identified. The pathologies presented are currently classified according to the stage of development at which the deviation from normal takes place, in: chromosomal sex abnormalities, gonadal sex abnormalities, and phenotypical sex abnormalities. Other congenital pathologies that frequently affect the male genital apparatus, such as cryptorchidism or hypospadias, will also be discussed.