We describe a female neonate with an oral teratoma showing bone, teeth, and
epidermis, but also epididymal (male) tissue. PCR amplification of Y-chrom
osomal DNA clearly showed male DNA from paraffin-embedded tumour tissue. Th
e girl had a normal female karyotype without abnormalities of the genital o
rgans. There are at least three hypotheses for the origin of teratomas: par
thenogenesis, incomplete twinning, and totipotent somatic-cell origin. This
case supports the hypothesis of an included dizygotic twin, and might cont
ribute to the elucidation of the pathogenesis of extragonadal teratomas.