Primary pulmonary lymphoma is a rare clinical neoplasm, and its atypical manifestation frequently leads to misdiagnosis. Here, we have reported a rare case of a 55-year-old man who presented with cough, dizziness, and fatigue. His chest computed tomography (CT) revealed diffuse ground glass pulmonary opacities. Bronchoscope lavage demonstrated lymphocyte predominance, while transbronchial biopsy indicated chronic inflammation. The administration of a broad-spectrum antibiotic regime supplemented with a high dosage of methylprednisolone was ineffective in improving the general condition of the patient, and the diffuse ground glass pulmonary opacities continued to worsen. CT-guided percutaneous lung biopsy confirmed the diagnosis of primary pulmonary lymphoma-diffuse large B-cell (PPL-DLBCL) without extrapulmonary involvement. The patient’s general condition improved with the systemic chemotherapy of CHOP. In the context of a systemic review of relevant literature, pulmonary lymphoma should be considered in the differential diagnosis of diffuse ground glass pulmonary opacities, and bronchoscopy is recommended for pathological diagnosis. Moreover, CT-guided percutaneous lung biopsy should also be adopted whenever necessary.