Aim and background: The aim is to illustrate the diagnostic challenge posed by pulmonary mucosa-associated lymphoid tissue (MALT) cell lymphoma, which can mimic interstitial lung disease (ILD). Representing 0.5–1% of lung neoplasia cases, primary pulmonary lymphomas, particularly MALT lymphomas, present diagnostic challenges due to varied imaging features and lack of specific biological markers. This case report highlights the diagnostic complexities when pulmonary MALT cell lymphoma mimics ILD, emphasizing the need for accurate histopathological confirmation. Case description: A 50-year-old female initially diagnosed and treated for ILD based on radiological findings, presented with worsening breathlessness and a dry cough. Examination revealed fine crackles in both lung fields, and imaging indicated reticular opacities suggesting ILD. Laboratory tests showed elevated serum lactate dehydrogenase and a positive ANA in autoimmune profiling. Despite treatment, symptoms worsened. Subsequent transbronchial biopsy confirmed pulmonary MALT cell lymphoma, prompting Rituximab therapy after multidisciplinary consultation. Conclusion: This case underscores the challenge of distinguishing between pulmonary MALT cell lymphoma and ILD solely based on radiological similarities. Accurate histopathological diagnosis through biopsies is pivotal in managing such cases effectively. Multidisciplinary collaboration facilitated a precise diagnosis and appropriate therapy, emphasizing its crucial role in managing complex conditions. Clinical significance: The case demonstrates the diagnostic complexity of differentiating pulmonary MALT cell lymphoma from ILD, stressing the necessity of histopathological confirmation. An accurate diagnosis significantly influences therapy prognosis and highlights the indispensable role of multidisciplinary collaboration in managing such rare cases.