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There are no clear estimates of the incidence rate of subjects presenting with ambiguous genitalia at birth, and only a proportion of them present a major challenge regarding male or female assignment.

However, it has been estimated to be approximately 1 in 4,500-5,500 [5].

Data are not available to determine the exact frequency of specific DSDs, while only a small fraction of those with DSDs require extensive multidisciplinary assessment to reach a recommendation for gender assignment.

The incidence rate among subjects with 46, XY to have a DSD has been estimated to be 1 in 20,000 births.

The broad term DSD, adopted at the Consensus Conference, has been generally accepted by many medical professionals but not universally by some patient and support groups [1,2].

Positive aspects about having a term include providing for scientific accuracy within a biological and medical context, bona fide genetic disorders facilitate access to healthcare and insurance, an umbrella classification helps in generating comprehensive and integrated models of care, at the same time it avoids confusion by not overlapping with conditions such as transgender, gender dysphoria and homosexuality, and it provides a framework for knowledge accumulation and research funding.

The evolution of diagnosis and care continues, while it is still impossible to predict gender development in an individual case with certainty.

Such decisions and decisions regarding surgery during infancy that alters external genital anatomy or removes germ cells continue to carry risk. Karger AG, Basel Disorders of sex development (DSDs) were defined as congenital conditions within which the development of chromosomal, gonadal and anatomic sex is atypical at the Chicago Consensus Meeting in 2005 [1].

Perceptions and the approach to the diagnosis and care of individuals with DSDs continuously change.

Data remain inadequate to address major concerns including the assignment of male or female sex, predictors of gender identity development, surgical issues regarding timing and consent and the best possible fertility preservation measures.

It is clear than gains in perceptions, approach and care since 2006 need to be reviewed and considered.

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