Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracranial tumors of neuroglial origin, mostly located in the supratentorial regions and particularly in the temporal lobe. Few cases of DNETs in the hypothalamus have been described. The authors present the case of a DNET in the hypothalamus. The 5-year-old girl with complaints of limb and gelastic seizures was admitted to the neurosurgical department of Xiangya Hospital. Neurological examination findings were unremarkable. MRI showed isointensity without significant enhancement on T1- and T2-weighted images. The lesion exhibited clearly defined borders on the sagittal, coronal, and axial images. The preliminary diagnosis was hypothalamic hamartoma (HH); however, the lesion was surgically removed, and histopathological examination confirmed the diagnosis of a DNET. Hypothalamic DNETs are extremely rare. Based on their clinical manifestation and imaging, DNETs are easily misdiagnosed as HHs. Diagnoses apart from HHs must be entertained when a hypothalamic lesion is being investigated.