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Usually, it is caused by unequal crossing over between X and Y chromosomes during meiosis in the father, which results in the X chromosome containing the normally-male SRY gene.

When this X combines with a normal X from the mother during fertilization, the result is an XX male.

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Rarer causes include mutation in an autosomal or X chromosomal gene or undetected mosaicism with a Y-bearing cell line.

This syndrome occurs in approximately four or five in 100,000 individuals, making it less common than Klinefelter syndrome.

Males typically have one X chromosome and one Y chromosome in each diploid cell of their bodies. XX males have two X chromosomes, with one of them containing genetic material from the Y chromosome, making them phenotypically male; they are genetically female but otherwise appear to be male.

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