Hypothalamic hamartomas are rare congenital gray matter heterotopias associated with a triad of gelastic seizures, precocious puberty, and developmental delay. Gelastic seizures typically have a childhood onset and are drug-resistant, with scalp electroencephalogram (EEG) having limited utility to identify abnormalities. On neuroimaging with brain MRI, the hamartomas are usually nonenhancing, are nearly isointense relative to the adjacent gray matter on all sequences, and are not associated with abnormal restricted diffusion.