Xanthogranuloma of sellar region is uncommon and difficult to differentiate from craniopharyngioma, Rathke's deft cyst and other sellar lesions by imaging findings. In this article, we report a rare case of sellar xanthogranuloma in a 49-year-old woman who presented with headache and visual defect. Computed tomography (CT), magnetic resonance imaging (MRI), and partial surgical excision were performed and pathology confirmed the diagnosis. Postoperative follow-up series of imaging studies demonstrated the residual tumor evolution in various size and partial response to the radiotherapy. Current evidence regarding to the clinicopathologic and radiological features of sellar xanthogranuloma will be summarized, along with discussion on its main differential diagnoses.