Evans syndrome (ES) is an autoimmune disease characterized by concurrent or sequential occurrence of autoimmune hemolytic anemia, immune thrombocytopenia and less frequently autoimmune neutropenia. ES is a rare situation that represents up to 7% of autoimmune haemolytic anaemia (AIHA) and around 2% of immune thrombocytopenia (ITP). The syndrome is characterized by a chronic, relapsing, and potentially fatal course requiring long-term immunosuppressive therapy. Treatment is based on corticosteroids and immunoglobulin injections depending on how the disease begins. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. This syndrome often occurs with other autoimmune diseases, such as systemic lupus erythematosus (SLE), common variable immunodeficiency (CVID), and autoimmune lymphoproliferative syndrome (ALPS).