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Systemic mastocytosis is a rare disease and characterized by excessive mast cell accumulation in one or multiple organs. One subtype of systemic mastocytosis is systemic mastocytosis-associated clonal hematological non-mast cell lineage disease (SM-AHMND), which indicates concurrent evolution of two separate clonal entities, one consisting of mast cells and one as a second hematological as well as non-mast cell origin disease. When SM-AHMND is diagnosed, bone marrow examination is essential for the initial approach, because marrow is almost universally involved in adult mastocytosis and it facilitates detection of a second hematological neoplasm. Myeloid neoplasm is reported to be the most prevalent associated clonal hematological non-mast cell disease. Treatment strategy and outcome for SM-AHMND is dependent on hematological non-mast cell lineage disease. Herein, we have presented a case report of diffuse large B cell lymphoma coexisting with systemic mastocytosis where the patient underwent successful chemotherapy leading to extended survival duration.