Catatonia is a neuropsychiatric syndrome that occurs with several psychiatric and systemic medical conditions. Central nervous system abnormalities are believed to increase the risk of catatonia, with several cases reported in patients with neurodevelopmental and neurodegenerative disorders. Lissencephaly is a spectrum of neuronal migration disorders characterized by poorly defined or absent gyri and sulci, abnormally thick cortex, and a broad array of histopathological changes. Lissencephaly has a range of etiologies, clinical signs, symptoms, and degrees of severity. It is associated with comorbidities, such as epilepsy, visual, cardiac, and skeletal problems, as well as premature mortality.In this article, we describe the case of a patient with lissencephaly who developed catatonia at 20 years of age. He was minimally responsive to treatment with a high dose of lorazepam, and catatonia subsided with the use of electroconvulsive therapy. This is the first case in our knowledge to describe the occurrence of catatonia among patients with lissencephaly and its treatment with electroconvulsive therapy.