We present a report of a stillborn female of mixed ancestry with congenitally short pancreas, polysplenia, congenital heart defect, normal bronchial branch pattern, and interruption of the inferior vena cava to add to the growing numbers (15) of this malformation complex in the literature. Additional manifestations were congenital hydrocephalus and absence of intestinal malrotation. A recent poster presentation at the Second International Workshop on Fetal Genetic Pathology in Montana by Drut et al. underlined the importance of reporting these cases and of the pathological examination required ["Abnormal Spleen Lobulation and Short Pancreas," in Blastogenesis-Normal and Abnormal. New York: Wiley-Liss, Inc. for the National Foundation-March of Dimes, 1993].