Science and Education Publishing American Journal of Medical Case Reports 2374-216X 3 2 2015 01 23 Systemic Lupus Erythematosus Revealing Splenic Marginal Zone Lymphoma. A Case Report 40 41 EN Bel Feki N Internal Medicine Department. University hospital of La Rabta, Tunis. Tunisia Smiti Khanfir M Said F Hamzaoui A Ben Salem T Ben Ghorbel I Lamloum M Ben Romdhane N Houman MH AJMCR2015326 10.12691/ajmcr-3-2-6 2014 12 04 2015 01 20 2015 01 23 Autoimmune manifestations are common in splenic marginal zone lymphoma (SMZL) and are sometimes the presenting feature of the disease. We report Herein the case of a 72-year old woman presenting with systemic lupus erythematosus revealing SMZL. To our knowledge, there are only 3 case reports of SLE associated to SMZL. In case of authentic autoimmune disease with unusual clinical features Physician awareness is recommended, to rule out associated lymphoproliferative syndrome.