A 71-year-old male presented with an isolated well-enhanced sellar lesion accompanied by hypopituitarism, diagnosed preoperatively as a pituitary adenoma, meningioma, or metastatic brain tumor. However, histological examinations yielded a diagnosis of neuroblastoma. Primary sellar neuroblastoma in the elderly is very rare. We therefore describe this case of primary sellar neuroblastoma, mimicking common pituitary tumor, and review the literature. There have so far been only nine reported cases of primary sellar neuroblastoma in the English literature. All reports like the present case, demonstrated similar neuroimaging of a “dumbbell-shaped extension in the sellar region.” Moreover, the tumors may exhibit characteristic features, such as rapid tumor growth, hypopituitarism, or oculomotor nerve palsy, and these findings may represent helpful signs for the diagnosis of primary sellar neuroblastoma.