Abstract
Usually, the distinction between male and female is considered absolute, allowing for instantaneous sex assignment at birth. This process is based primarily on external genitalia, which are typically classified as either male or female. However, in rare instances, ambiguity in external genitalia prevents immediate sex assignment. This necessitates further clinical evaluation commonly known as Disorders of Sex Development (DSD).
Among various types of DSDs, Ovo testicular DSD is a particularly rare condition, with approximately only 500 cases reported worldwide. Among various types of DSDs, a case of Ovo testicular DSD with a 46, XX/46, XY karyotype has been presented in this article. A comprehensive analysis, including detailed investigation and diagnostic evaluation, is provided. Additionally, we discuss various management options, emphasizing the clinical challenges and considerations associated with this rare disorder.
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