Autoimmune manifestations including autoimmune hemolytic anemia (AIHA) are often described in association with splenic marginal zone lymphoma (SMZL) . Moreover, AIHA has been recently reported as a potential risk factor of poor outcome for SMZL patients . Among the different available therapeutic options, rituximab monotherapy has been recently shown to be extremely effective in SMZL . On the other hand, this monoclonal antibody plays a key role in the treatment of steroid-refractory AIHA . More specifically, although rituximab has been recently included among the possible strategies to treat AIHA secondary to chronic lymphocytic leukemia , very few cases describing its use in the course of SMZL-related AIHA have been described so far .