Hypophysitis is an inflammation of the pituitary gland that originates directly within the pituitary, primary forms, or from neighboring or systemic diseases, secondary forms. Primary hypophysitis comprises three main histologic types granulomatous, xanthomatous, and lymphocytic. Autoimmune hypophysitis is rare but increasingly recognized. It offers a fascinating model to study the effect of pregnancy on autoimmune diseases and the interactions between nervous, endocrine, and immune systems. Furthermore, the main diagnostic issue with autoimmune hypophysitis is the differentiation between the rare autoimmune hypophysitis and the overwhelmingly more common pituitary tumors. The treatment of autoimmune hypophysitis is, at the moment, only symptomatic. It includes reduction of the pituitary mass and replacement of the defective hormones. Patient management can be improved when a reliable serologic test that identifies the autoimmune nature of the pituitary disease becomes available. Much remains to be learned about the natural history of autoimmune hypophysitis. Establishment of experimental models in mice and of a disease registry in humans can greatly advance the field.