Hypothalamic hamartoma is a rare, benign, heterotopic developmental malformation that consists of a mixture of normal neurons and glial cells. These developmental lesions arising from the tuber cinereum and inferior hypothalamus are usually diagnosed presenting with symptoms of precocious puberty, an intractable epilepsy syndrome, or disruptive behavioral and intellectual deterioration. Hypothalamic hamartomas attached to the mammillary region of the posterior hypothalamus are associated with epilepsy manifested as gelastic seizures, with most cases being resistant to antiepileptic drugs. Drug-resistant gelastic epilepsy requires early diagnosis and surgical treatment. Owing to the characteristics of hypothalamic hamartomas, surgical treatment—including resection and disconnection techniques—is primarily preferred. However, several other minimally invasive techniques are also recommended, each with variable success, including interstitial radiosurgery, stereotactic radiofrequency thermocoagulation, and magnetic-resonance–guided stereotactic laser thermocoagulation. This article aims to provide a general overview of hypothalamic hamartomas associated with epilepsy.