A female term infant was born with multiple bony and facial abnormalities involving the mandible, palate, tongue, nasopharynx, vertebrae, ribs and brain. Death occurred at 1 week and a rare combination of forebrain anomalies, were found in a big head, with complete agenesis of the corpus callosum with duplication of the infundibulum, pituitary, anterior third ventricle and aqueduct. The CAT scan was diagnostic of agenesis of corpus callosum. Incomplete twinning or incompetent neural induction with genetic and epigenetic factors have been suggested as possible mechanisms responsible for this rare malformation.