Colpocephaly is a rare congenital brain malformation resulting in disproportionately large occipital horns of lateral ventricles compared to frontal ones resulting from corpus callosal and other white matter developmental arrest. Colpocephaly can be associated with partial or complete agenesis of the corpus callosum, chiari malformations, lissencephaly, and microcephaly. Colpocephaly can present with headache, seizure disorder, visual impairment, movement abnormalities and muscle spasms or can even be asymptomatic. We report a case of a 21-year old male with colpocephaly presenting as a status epilepticus (SE) as an initial symptom along with incidental cardiac anomaly OS ASD (Ostium secundum atrial septal defect) found on routine screening.