A nine-month old boy was brought to our hospital with a complaint of growth retardation. On cerebral magnetic resonance imaging examination, giant clefts resulting in the connection of lateral ventricles with subarachnoidal spaces were detected in both cerebral hemispheres, and interpreted as bilateral giant open-lip schizencephaly. Associated anomalies were noted on cerebral magnetic resonance imaging examination. Bilateral thalami were located inferiorly. The caudate nuclei were observed but their configuration was disrupted. The other parts of the basal ganglia including globus pallidi and putamina were absent. We report a case of bilateral giant open-lip schizencephaly with accompanying basal ganglia anomalies. © 2009 Avcu et al; licensee Cases Network Ltd.