A two-year-old girl was found to have a ring 14 chromosome: [46, XX, r(14) (P13 q32.3)]. Her development, including verbal ability, was retarded, her CT scan displayed a low density area anterior to the left temporal lobe, and she suffered from complex partial seizures. Focal central nervous system abnormalities may be present in patients with ring 14 chromosome, and their seizures are not exclusively of the primary generalized type. This is the first case with ring 14 chromosome and complex partial seizures.