We describe two children with the velocardiofacial syndrome and benign part
ial-onset seizures. Both presented with slight dysmorphic traits, mild to m
oderate mental delay, and high-arched palate. A cardiac defect was present
in only one of them. In each patient, sporadic rolandic or occipital partia
l-onset seizures with the clinical and electroencephalographic features of
benign idiopathic childhood epilepsy manifested at age 3 and 5 years, respe
ctively. Treatment was started only in one patient, with complete seizure c
ontrol. These two cases show that benign partial epilepsy can be a componen
t manifestation of the central nervous system-related symptoms of the veloc
ardiofacial syndrome. (C) 2001 Wiley-Liss, Inc.