Hypophysitis is a broad term used to describe conditions leading to inflammation of the pituitary gland and the pituitary stalk. It may develop as a primary condition or secondary to other diseases. Hypophysitis is classified based on aetiological, anatomical, and histological criteria. Clinical symptoms result from enlargement of the pituitary gland, hormonal deficiencies, diabetes insipidus, and hyperprolactinemia. Histopathological verification of tissue samples from a pituitary biopsy remains the gold standard in diagnosing hypophysitis. However, due to the invasiveness and risk of the procedure it is rarely performed. The diagnosis is based mainly on clinical presentation, laboratory tests, and imaging. The rarity of the disease and the deficit in reliable data result in a lack of clear guidelines in the treatment of hypophysitis. The basic therapy relies on hormonal replacement. High doses of steroids are the first-line treatment of symptoms caused by mass effect in sella and compression of surrounding structures. In steroid-resistant patients or in cases of inacceptable sides effects, treatment with other immunosuppressant drugs was administered with success. The course of the disease varies: some patients present remission, in other cases hypophysitis leads to fibrosis and atrophy of the pituitary gland, which is reflected in persistent hormonal deficiencies and images of an empty sella. The objective of this article is to present the most important information: the epidemiology, clinical image, diagnostic procedures, and treatment of primary hypophysitis, in order to allow better understanding of this disease and implementation of proper management. Posttraumatic and immunotherapyrelated hypophysitis are also briefly characterised.