Hypothalamic malformation tumor is an extremely rare congenital developmental anomaly that often manifests as dementia epileptica, central precocious puberty, and cognitive dysfunction. In recent years, new techniques and tools for the treatment of hypothalamic malformation tumors have emerged, but their pathogenesis is still poorly understood, and there are many different opinions on how to choose the appropriate surgical plan for hypothalamic malformation tumors with different clinical manifestations. In this article, we discuss the pathogenesis of hypothalamic malformation tumors based on clinical experience and relevant literature, and introduce the progress of clinical detection and various surgical treatments as well as their applicable principles. Dementia attacks are a specific clinical manifestation of hypothalamic malformation tumor, and they mostly appear in the early stage of the disease. As the disease progresses, dementia seizures can evolve into various complex types of seizures. fMRI and intracranial EEG monitoring findings suggest that most epilepsy originates from the hypothalamic malformation tumor itself. Dementia seizures are mostly drug-refractory epilepsy, for which surgery is the treatment of choice. Other adjuvant treatments (e.g., ketogenic diet) are less effective. At present, the mechanisms and details of surgical treatment remain complex issues. This review discusses the progress of clinical research on hypothalamic malformation tumors based on clinical experience and analysis of relevant literature.