Hypothalamic hamartomas (HHs), although rare, has raised much interest due to the uniqueness of their clinical symptoms, the mystery of the subcortical-cortical networks involved in their pathogenesis, and the unparalleled complexity posed by their surgical treatment as a lesion-related epilepsy.Paillas, Roger, and colleagues in 1969 were the first to describe the clinical, radiologic, and histologic presentation of "epileptic" HH 1 and to resect the HH in order to treat the associated epilepsy.1 Since then, concepts of the electroclinical expression of epilepsy observed in association with HHs, and the available therapeutic approaches, have evolved rapidly and dramatically. The development of different surgical approaches, and of the concepts about the role of the HHs in relation to the epilepsy, in this typically drug-resistant condition, 2 has gone through a series of cycles. In 1993, Cascino and colleagues reported evidence of seizure onset involving the temporal (and/or frontal) lobe leading to neocortical resection following the traditional electroclinical mapping scheme of the epileptogenic zone with surgical implantation of intracranial electrodes (without electrode placement into the HH lesion). This strategy led to repeated failures. 3 In 1995, Claudio Munari, using stereoencephalography (SEEG), recorded ictal discharges in the HH, 4 thus demonstrating the role of the HH itself as an intrinsically epileptogenic lesion. Simultaneously in support of this hypothesis came the demonstration of hyperperfusion of the HH with ictal single-photon emission computed tomography (SPECT) by Kuzniecky and colleagues. The concept of an essentially subcortical epilepsy, with the epileptogenic zone (EZ) being centered on the HH itself, paved the way for lesion resection becoming a standard treatment for HHs that were associated with drug-resistant epilepsy. Such a lesional approach tended to require a relatively simple preoperative workup, notably without intracranial recording in the majority of cases. Small series