Colpocephaly describes that occipital horn enlargement of the ventricular system and the frontal horn status is normal. Corpus callosum agenesis is most common structural abnormality of brain. Coexistence of corpus callosum agenesis and colpocephaly have been shown in many studies. Cases are affected in varying degrees from severe neurological damage to healthy subjects due to the corpus callosum agenesis. Seven years old girl who had convulsion three months ago, admitted to our hospital. It was learned that the first convulsion and had no disease before of the patient. Colpocephaly and corpus callosum agenesis were reported at the magnetic resonance imaging. This case report was presented for emphasize to so important of imaging methods in convulsion etiology with before healthy cases and also was presented to emphasize the association corpus callosum agenesis with many clinical situations.