Evans syndrome (ES) is a chronic hematological disorder characterized by autoimmune hemolytic anemia and immune-mediated thrombocytopenia that often requires profound and long-term immunosuppression. Only a few small case series or single case studies of autologous and allogeneic hematopoietic stem cell transplantation (HSCT) in patients with ES have been reported in the literature, with long-term remissions being observed after both autologous and allogeneic HSCT. Patients with ES suffering from refractory disease, multiple relapses and serious disease-related complications should be offered allogeneic HSCT, which is the only treatment with curative potential. Autologous HSCT might be preferable in patients with serious pre-existing comorbidities lacking an HLA-identical donor. Owing to the rarity of this disease and the small number of patients receiving HSCT for ES, prospective controlled studies on this approach are not available. A prospective registration of patients transplanted for ES would allow the development of optimal transplant strategies.