An autopsy case of short rib-polydactyly syndrome, type II (Majewski type) complicated by anisosplenia is reported, and some discussion has been made from the fetopathological viewpoint on the charactaristic findings and concomitant malformations of this syndrome with regard to a total of 23 cases, including our case and 22 cases reported in literature.Presented at the 24th Annual Meeting of the Congenital Anomalies Research Association of Japan July 6-7, 1984