Hemimegalencephaly (HME) is a rare cortical developmental anomaly that can be defined as abnormal neural and glial proliferation localized to all or part of a cerebral hemisphere. Tuberous sclerosis complex (TSC), is an autosomal dominant, multisystem disease that involves most frequently the central nervous system, skin, heart, kidneys, eyes. Herein, we reported the magnetic resonance imaging findings of an unusual case of focal megalencephaly with TSC.