The epidemiology of congenital malformations in Japan was reviewed based on National Vital Statistics. A six-fold increase was noted in the frequency of malformations as cause of fetal death attended by physician from 538 out of 151,338 (0.36%) in 1952 to 2,476 out of 122,273 (2.02%) in 1972. The increase in the ratio of malformations was noted as statistically significant both in spontaneous and artificial fetal deaths as well as in both middle and late fetal deaths. Anencephalus was noted as a major malformation responsible for such increase in fetal death: 88 or 0.33% in 1952 and 837 or 3.42% in 1972. The increase in musculo-skeletal malformations and malformations due to chromosomal abnormality was noted as statistically significant in malformations in early neonatal infant deaths and infant deaths respectively. Possible cause for the increase of anencephalus were discussed by reviewing relevant literature, and a need for systematic epidemiological study in this country was emphasized.