BACKGROUND: Epilepsy is a chronic disease that affects millions of people around the world generating great expenses and psychosocial problems burdening the public health in different ways. A considerable number of patients are refractory to the drug treatment requiring a more detailed and specialized investigation to establish the most appropriate therapeutic option. Insular epilepsy is a rare form of focal epilepsy commonly drug resistant and has much of its investigation and treatment involved with the surgical management at some point. The insula or the insular lobe is a portion of the cerebral cortex located in the depth of the lateral sulcus of the brain; its triangular in shape and connects with the other adjacent lobes. The insular lobe is a very interesting and complex portion of the brain related with different functions. Insula in Latin means Island and was initially described in the 18 century but its relation with epilepsy was first reported in the 1940-1950s. Insular lobe epilepsy is generally difficult to identify and confirm due to its depth and interconnections. Initial non-invasive studies generally demonstrate frustrating or incoherent information about the origin of the ictal event. Technological evolution made this pathology to be progressively better recognized and understood enabling professionals to perform the correct diagnosis and choose the ideal treatment for the affected population. METHODS: A literature review was performed using MEDLINE/PubMed, Scopus, and Web of Science databases. The terms epilepsy/epileptic seizure of the insula and surgical treatment was used in various combinations. We included studies that were published in English, French, or Portuguese; performed in humans with insular epilepsy who underwent some surgical treatment (microsurgery, laser ablation, or radiofrequency thermocoagulation). RESULTS: Initial search results in 1267 articles. After removing the duplicates 710 remaining articles were analyzed for titles and abstracts applying the inclusion and exclusion criteria. 70 studies met all inclusion criteria and were selected. CONCLUSION: At present, the main interests and efforts are in the attempt to achieve and standardize the adequate management of the patient with refractory epilepsy of the insular lobe and for that purpose several forms of investigation and treatment were developed. In this paper, we will discuss the characteristics and information regarding the pathology and gather data to identify and choose the best therapeutic option for each case.