The occurrence of multiple malignancies in the same patient being synchronous or metachronous is a rare event. The incidence of multiple malignancies varies with age, sex, geographic origin, and site and type of tumors. The pathogenetic etiology may be multifactorial and include genetic predisposition, immunodeficiency, radiation therapy, chemotherapy and various infectious agents. It is crucial to recognize synchronous malignancies because course of treatment and management is difficult. The synchronous occurrence of pulmonary squamous cell carcinoma and ileal diffuse large B-cell lymphoma (DLBCL) is not reported in the Indian medical literature until today; hence, we publish this case for its rarity.