Focal epilepsy originating from the insular cortex is rare. One reason is the small amount of cortical tissue compared with other lobes of the brain. However, the incidence of insular epilepsy might be underestimated because of diagnostic difficulties. The semiology and the surface EEG are often not meaningful or even misleading, and elaborated imaging might be necessary. The close connections of the insular cortex with other potentially epileptogenic areas, such as the temporal lobe or frontal/central cortex, is increasingly recognized as possible reason for failure of epilepsy surgery for temporal or extratemporal seizures. Therefore, some centers consider invasive EEG recording of the insular cortex not only in case of insular epilepsy but also in other focal epilepsies with nonconclusive results from the presurgical work-up. The surgical approach to and resection of insular cortex is challenging because of its deep location and proximity to highly eloquent brain structures. Over the last decades, technical adjuncts like navigation tools, electrophysiological monitoring and intraoperative imaging have improved the outcome after surgery. Nevertheless, there is still a considerable rate of postoperative transient or permanent deficits, in some cases as unavoidable and calculated deficits. In most of the recent series, seizure outcome was favorable and comparable with extratemporal epilepsy surgery or even better. Up to now, the data volume concerning long-term follow-up is limited. This review focusses on the surgical challenges of resections to treat insular epilepsy, on prognostic factors concerning seizure outcome, on postoperative deficits and complications. Moreover, less invasive surgical techniques to treat epilepsy in this highly eloquent area are summarized.