A 2-month-old infant girl died suddenly as a result of torsion of the uterine adnexa. The infant was found unresponsive in bed and was pronounced dead shortly after her arrival at a hospital. There were no antecedent signs of illness. At autopsy, the right ovary and right fallopian tube were twisted and were dark purple, swollen, and necrotic. The right ovary was enlarged by a follicle cyst 4 cm in diameter, which likely precipitated the torsion. The mechanism of death was unclear but may have resulted from the release of cytokines produced in response to necrotic adnexal tissue. Fatal uterine adnexal torsion has been reported rarely in infants; in all those cases there were antecedent symptoms. Torsion of the uterine adnexa should be included in the differential diagnosis of sudden death in infancy.