© 2017 Elsevier Inc. A hypothalamic hamartoma consists of hyperplastic heterotopic tissue growing in a disorganized fashion. These lesions occur in about one per 50,000 to 100,000 people. Hypothalamic hamartomas can cause intrinsic epileptogenesis leading to gelastic seizures. Surrounding cortical structures may also develop secondary epileptogenesis. Persistent seizures caused by hypothalamic hamartomas can be debilitating and result in significant cognitive and behavioral impairment. Early recognition and treatment is important in controlling seizures and in preventing further cognitive deterioration. Some patients experience improved cognition and behavior following early treatment, suggesting that hypothalamic hamartomas represent a reversible epileptic encephalopathy. The outcome of epilepsy associated with these lesions has significantly evolved with the availability of new treatment techniques and an improved understanding of its pathogenesis. Increasing evidence supporting the role of hypothalamic hamartomas as a cause of gelastic seizures and secondary epileptogenesis has led to more frequent use of surgery as the definitive treatment. Several minimally invasive procedures have been devised, including neuroendoscopic approaches and different stereotactic radio and laser ablation techniques. Each of these techniques can lead to unique adverse events. We review the various classification schemes used to characterize hypothalamic hamartomas and the recommended surgical approaches for each subtype. We also review the literature for currently available treatment modalities and compare their efficacy in controlling seizures and their safety profiles.