We report an association of trigonocephaly and thumb hypoplasia in a 6
.5-year-old boy, diagnosed as Baller-Gerold syndrome. In addition to c
raniosynostosis and radial limb defect, which are constant in this syn
drome, our patient presents two unusual features: the first is an epid
ermal nevus and the second is an agenesis of the middle portion of cor
pus callosum. This unique type of callosal agenesis in the context of
a polymalformative disorder supports the hypothesis that partial agene
sis of corpus callosum may be due to an event occurring before the 12t
h week gestation with continued development of the midline structures.