Xanthogranulomatous hypophysitis is a rare and poorly understood heterogeneous disease. The etiology of xanthogranulomatous hypophysitis remains unknown at this time and there are no clear presurgical diagnostic markers making prospective studies difficulty at this time. Our patient demonstrated pathology most consistent with xanthogranulomatous hypophysitis/xanthogranulomatous hypophysitis . In addition to his pituitary lesion, he was found to have lesions in his ureter causing hydronephrosis and in his spinal cord. There are other reports of xanthomatous changes in extrapituitary locations as mentioned previously, though this is a rare occurrence. Further understanding of xanthogranulomatous hypophysitis is required to better learn how to treat these patients.