Xanthogranulomas are rare intracranial lesions with controversial etiology. The sellar location is exceedingly rare. Here we report a clinical case and a review of the English-language literature of histologically confirmed xanthogranulomas in order to furnish useful tools in diagnosis and management of this unusual disease. We performed an English-language literature MEDLINE search for the last 18 years and analyzed the reports of the published series and the present case. The clinical, radiological, pathological features and outcome of the published cases of Xanthogranuloma have also been compared with the traits of Craniopharyngioma and Rathke Cleft Cyst. The data collection has been hindered by the lack of important details in the published series. The available clinical and radiological data have been reported in Table 1 (28 papers for a total of 59 patients reported). A clinical-radiological comparison among common pathologies of the sellar-parasellar region has been performed in Table 2. Endocrine impairment was a common finding in the clinical presentation and it was often worsened by surgery. Natural history of Xanthogranuloma is similar to other benign pathologies of the sellar area, but some typical features might help in distinguishing it before the pathological exam.