Hypothalamic hamartomas (HH) are lesions that arise in the ventral hypothalamic region. The syndrome is commonly associated with a spectrum of clinical, endocrine, and neuropsychological comorbidities including refractory epilepsy, precocious puberty, and rage behaviors. Additionally, Hypothalamic Hamartoma Syndrome may include the evolution of an epileptic encephalopathy with developmental regression, psychiatric and behavioral comorbidities.