Hypothalamic hamartomas are nonneoplastic malformations of fetal brain development, frequently causing severe drug-resistant epilepsy. The imaging characteristics of their variable extension and their relation to the hypothalamus, the third ventricle, and the basal cisterns can be more clearly assessed only since the introduction of magnetic resonance imaging, which in turn has influenced the development of several surgical approaches and their indications. The aim of surgery has been to resect or disconnect this epileptogenic malformation, either through one of the different craniotomy approaches from below or above the third ventricle or via the endoscopic transventricular route. Stereotactic ablation procedures have also been developed, such as radiofrequency thermocoagulation or MRI-guided laser ablation. In this chapter, an overview is given of the different craniotomy approaches, either reaching from the skull base or from above the third ventricle. The endoscopic transventricular technique for hamartoma disconnection, originally developed and frequently practiced at the author’s institution, is presented in more detail. At the end of the chapter, the various approaches and their suitability in relation to each individual case are discussed.