Most individuals with the rare 46,XX male ''syndrome'' arise due to an
unequal interchange between Xp and Yp termini during paternal meiosis
. The pattern of Y-sequences in these patients varies considerably, bu
t very few cases have been reported showing only SRY. The phenotype in
these patients is also variable ranging from severe impairment of the
external genitalia through hypospadias and/or cryptorchidism to occas
ional normal male phenotype. We report a Mexican 46,XX male patient wi
thout genital ambiguities in whom DNA analysis showed the presence of
SRY and the absence of ZFY. We conclude that in this case SRY alone wa
s enough for complete male sexual differentiation. (C) 1995 Wiley-Liss
, Inc.