Schizencephaly is a very rare congenital birth defect. It is characterized by a cortical brain malformation that manifests as a grey-matter-lined cleft extending from the ependyma to the pia mater. It is a rare condition, and few cases have been reported in the literature. The exact cause is unknown. Herein, we report a case of an infant presenting with left side hemiparesis. The CT scan of her brain revealed right fronto-temporal and left parieto-temporal open-lip schizencephaly; thus, urgent referral to a pediatric neurologist was made for early intervention.