Acquired hemophilia is a rare hemostatic disorder caused by the development of neutralizing autoantibodies (inhibitors) against coagulation factors, most commonly directed against factor VIII (FVIII). In this paper, most terms refer to acquired hemophilia caused by inhibitors against FVIII, known as acquired hemophilia A (AHA), although there are very rare cases involving acquired inhibitors against other coagulation factors. These inhibitors lead to a reduction in FVIII activity, resulting in an increased tendency for bleeding with high morbidity and mortality. Acquired hemophilia has a low incidence of approximately 1.5 cases per million people, and its incidence increases with age. The disease is often associated with various conditions, including autoimmune and malignant diseases, pregnancy, and the postpartum period, and it can also be idiopathic. Recognizing early symptoms and triggers, promptly establishing a diagnosis, and initiating therapy are crucial for managing this complex condition. The clinical presentation of acquired hemophilia involves the sudden and unexpected onset of bleeding, either spontaneously or after an invasive procedure, in individuals without a personal or family history of bleeding. Early diagnostic procedures are critical and complex. Characteristic laboratory features of acquired hemophilia include prolonged activated partial thromboplastin time, reduced FVIII activity, and the presence of autoantibodies against FVIII. The primary therapeutic goals are to control bleeding with hemostatic therapy and to eliminate inhibitors with immunosuppressive therapy, along with treating any underlying disease. An individualized approach to each patient is essential, as some patients have concurrent elevated risks for thrombotic and infectious complications.