A 52-year-old man presented in October 2016 with a 3-week history of easy fatigability, intermittent fever, cough, and dyspnea. He was diagnosed with hepatitis C virus-negative splenic marginal zone lymphoma (SMZL) in 2008 and had undergone splenectomy. Physical examination revealed mild hepatomegaly without lymphadenopathy, and the chest was clear on auscultation. Laboratory tests were unremarkable, except for mild anemia. Chest computed tomography (CT) was performed on suspecting relapse, which revealed multiple pulmonary nodules (A). Considering miliary mottling, he underwent evaluations for tuberculosis. His Mantoux test results were negative and bronchoalveolar lavage fluid did not show acid-fast bacilli. Polymerase chain reaction for tuberculosis and Mycobacterium growth culture were also negative. CT-guided biopsy of the lung nodules showed non-Hodgkin's lymphoma (C). The MIB-1 labeling index was 50–60% (D). The tumor cells were positive for cluster of differentiation (CD)20 (E) and negative for CD10 (F) and Bcl2, consistent with lung involvement as marginal zone lymphoma. He was administered rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone; after 4 cycles, chest CT revealed complete response (B). Our case highlights an unusual SMZL relapse pattern after splenectomy, emphasizing the need for re-biopsy in patients with rare lymphoma presentations.