There are numerous reports describing the pathology of the fetus and placen
ta in triploidy. Although gonadal pathology is described in many of these r
eports, consistent changes have not been noted nor is it clear whether geni
tal ambiguity can be considered part of the triploid phenotype. We present
a case of triploidy of probable diandric origin, in which there were dysgen
etic gonads with abnormal seminiferous tubules, nodules of undifferentiated
stroma, and focal absence of the tunica albuginea. As this finding was dis
tinctly unusual in our experience of triploid gonadal pathology, we reviewe
d the gonadal histology in 51 fetal and infant triploids examined in our au
topsy/embryopathology laboratory. The gonads were compared to age-matched n
ormal controls to determine if there was a specific gonadal pathology assoc
iated with triploidy and if there was any correlation of this pathology wit
h parental origin of the triploidy. Our review of the triploid gonads indic
ated that while minor, nonspecific changes were not uncommon, overtly dysge
netic gonads, as observed in the index case, are rare.