The main objective was to review the evidence for management of epilepsy associated with hypothalamic hamartomas. We performed a systemic review of the literature through July 2012 that studied patients with hypothalamic hamartomas and related epilepsy. Articles meeting selection criteria were rated according to the American Academy of Neurology classification of evidence scheme. Recommendations were linked to the strength of the evidence and as follows: (a) precocious puberty is associated more with the pedunculated type and epilepsy typified by gelastic seizures with the sessile form of hypothalamic hamartomas (class III); (b) significant behavioral and cognitive deficits are associated with patients with hypothalamic hamartomas (class III); (c) video electroencephalography (EEG) findings are extremely variable particularly across the different ages and do not affect surgical outcome (class III); d) various surgical techniques (transcallosal and endoscopic resection) resulted in 49% to 54% seizure freedom, 15% with a pterional approach as well as about 40% with radiosurgery (class III). © The Author(s) 2013.