Primary ovarian insufficiency (POI) is a complicated clinical syndrome characterized by progressive deterioration of ovarian function. Autoimmunity is one of the main pathogenic factors affecting approximately 10% to 55% of POI cases. This review mainly focuses on the role of autoimmunity in the pathophysiology of POI and the potential therapies for autoimmunity-related POI. This review concluded that various markers of ovarian reserve, principally anti-Müllerian hormone, could be negatively affected by autoimmune diseases. The presence of lymphocytic oophoritis, anti-ovarian autoantibodies, and concurrent autoimmune diseases, are the main characteristics of autoimmune POI. T lymphocytes play the most important role in the immune pathogenesis of POI, followed by disorders of other immune cells and the imbalance between pro-inflammatory and anti-inflammatory cytokines. A comprehensive understanding of immune characteristics of patients with autoimmune POI and the underlying mechanisms is essential for novel approaches of treatment and intervention for autoimmune POI.