Disorders of sex development comprise a heterogeneous group of heritable abnormalities of sexual determination and differentiation, formerly termed “intersexuality”. This includes chromosomal as well as monogenic disorders, which inhibit primarily genetic or endocrine pathways of normal sex development. The genes implicated in the complex process of development are expressed in early fetal life and almost all of them play roles in the development of other systems as well, this widespread expression of genes causes mulitsystem affection when these genes are mutated. Also among the genetic factors influencing development the enzymes and cofactors involved in steroid biosynthesis, predominantly driven by adrenals and gonads, which derive from the common urogenital ridge during early fetal development. In this study we selected 30 cases of DSD associated with an affection of another system. Among the selected cases, neurological affection was the most common associated anomaly, also noticed were cardiac, skeletal, dysmorphic features, growth affection and genitourinary systme affection. Cases were subjected to karyotype and FISH for those indicated.