Background: Out of the various malignant tumours originating from the lymphatic hematopoietic system, lymphoma is one such important entity. It is divided into Non-Hodgkin’s Lymphoma (NHL) and Hodgkin Lymphoma (HL) depending on its cell source. A very rare type of malignant variant of lymphoma is the primary splenic lymphoma, involving exclusively the spleen and splenic hilar lymph nodes. Moreover, splenic marginal zone lymphoma (SMZL) is even more infrequent. SMZL is an uncommon chronic B lymphocyte proliferative disease, which only accounts for about 1–2% of all non-Hodgkin’s lymphoma. The mean age of SMZL incidence is about 65 years. There is no known significant gender predominance. A quarter of patients with early diagnosed SMZL have known to have vague symptoms like abdominal pain and distention; and other patients may be accompanied by loss of weight, malaise, cachexia, splenomegaly, or other manifestations.
Conclusion: Although, a good prognostic outcome is what is usually expected from most patients of Splenic Marginal Zone Lymphoma who undergo splenectomise, an aggressive transformation leading to a worse direction cannot be ruled out. SMZL is very challenging to be diagnosed pre-operatively due to the lack of specificity in clinical presentation.