We evaluated brain abnormalities associated with myelomeningocele in infants. Between June 1995 and June 2008, 42 patients with myelomeningocele were treated in our hospital. Only 24 patients (13 males, 11 females, mean age 1.5 months, range 1 day - 11 months) were evaluated by both spinal and brain magnetic resonance imaging (MRI) and were enrolled in the study. Brain MRI revealed: hydrocephalus in 21 (87.5%) patients, all of whom required immediate shunting. Total agenesis of the corpus callosum was observed in 2 (8.3%) patients, partial agenesis was seen in 4 (17%) patients and 8 (34%) patients had dysplasia of the corpus callosum. Absence of the septum pellucidum was observed in 2 (8%) patients. Widening of the interhemispheric fissure and colpocephaly were noted in 10 (41%) and in 3 (12%) patients, respectively. Abnormal white matter maturation was observed in 2 (8%) patients. Small posterior fossa was observed in 18 (74%) patients, Chiari malformation in 16 (67%) patients, cerebellar and brain stem hypoplasia in 3 (12%) and 7 (30%) patients, respectively. MRI examination of the myelomeningocele site is not sufficient. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with myelomeningocele.