Ganglion cell tumors of the central nervous system (CNS) are uncommon. There have been few reports in the literature about ganglion cell tumors that arise from the spinal cord, pineal gland, cerebral hemisphere or cerebellum. We recently experienced a case of ganglioneuroblastoma that developed from the hypothalamus in 4-year-old boy. To the best of our knowledge, this is the first reported case of ganglioneuroblastoma in the hypothalamus. We report on this case and we present the neuroimaging and pathologic findings.