This report presents a patient with medically intractable startle epilepsy who was treated with surgery. The ictal onset zone was further localized to a part of the premotor cortex on the basis of intracranial electroencephalography recording and surgical resection of the epileptogenic zone was done. The motor area close to the epileptogenic focus was localized using electrical cortical stimulation and thus preserved. Patient has been seizure-free postoperatively (Engel Class I) during the 18-month follow-up. The patient illustrates that surgery can be an option in patients with medically intractable startle epilepsy with well-defined unilateral epileptogenic zone.