Schizencephaly is a rare central nervous system (CNS) malformation secondary to neuronal migration defects. The pathogenesis is complex and is secondary to environmental and genetic factors. Clinically, the majority of patients present with varying degrees of motor and psychological disturbances. Imaging plays a cornerstone in the diagnosis by identifying the characteristic lesional features and recognising other associated abnormalities such as an absent septum pellucidum and corpus callosum dysgenesis. Here, we present a male paediatric case who presented with an interestingly asymptomatic unilateral right closed-lip schizencephaly and review the aetiology, clinical presentation and imaging characteristics of the disease and associated literature.