BACKGROUND: Xanthogranulomas are uncommon granulomatous entities, and xanthogranulomas occurring in the sellar region are extremely rare, with a limited number of cases reported in the literature. Diagnosis, management, and prognosis of sellar xanthogranulomas are poorly understood. METHODS: We retrospectively reviewed 14 patients with histologically proven sellar xanthogranulomas treated at our institution between January 2006 and July 2016. Clinical manifestations, radiologic features, operative records, and follow-up outcomes were analyzed. RESULTS: There were 9 male and 5 female patients with a mean age of 29.2 years. Clinical manifestations included headache (n =12; 85.7%), visual deficiencies (n = 10; 71.4%), and endocrine disturbances (n = 6; 42.9%). On magnetic resonance imaging, sellar xanthogranulomas revealed isointensity to hyperintensity on T1-weighted imaging and hyperintensity or heterogeneous intensity on T2-weighted imaging with variable enhancement. Gross total resection of the lesion was achieved in 10 cases, and subtotal resection was achieved in 4 cases. Post-operatively, headache was relieved in all cases; transient diabetes insipidus was observed in 2 cases. There were no procedure-related complications. During a mean follow-up period of 52.9 months, visual deficiencies were improved in 7 (70.0%) cases, whereas endocrine disturbances were improved in only 2 (33.3%) cases. No recurrence or regrowth of the residual lesions was observed. CONCLUSIONS: Sellar xanthogranuloma should be considered in the differential diagnosis of lesions in the sellar region. Some radiologic characteristics, including hyperintensity on T1-weighted imaging, heterogeneous hyperintensity on T2-weighted imaging, variable enhancement, and absence of calcification, can assist in making the preoperative diagnosis. Surgical outcomes are favorable.