ObjectiveTo improve the diagnosis and treatment of multiple systemic sarcoidosis (MSS) and avoid misdiagnosis. MethodsTo analyze the diagnosis and treatment of a MSS patient misdiagnosed as lymphoma. Related literatures were also reviewed. ResultsThe patients' clinical manifestations were not specific including cough and stethocatharsis. Lung and thoracic lymph nodes were most commonly involved in MSS. MSS was characterized by symmetrical lymph nodes enlargement in the bilateral lung hilus and/or mediastinum. The enlarged lymph nodes had a clear boundary and showed homogeneous enhancement. Symmetrical fluorodeoxyglucose (FDG) uptake in the hilar and/or mediastinal node was a typical finding of sarcoidosis on FDG PET/CT. Mucosal inflammation and mucosal nodules could be seen in the bronchoscope. Sarcoidosis was characterized by the presence of noncaseating groanulomas histologically. Hormonal therapy was effective for MSS. ConclusionSarcoidosis is a kind of disease involving multiple systems and organs with unknown etiology. The clinical manifestation of sarcoidosis is nonspecific,so it's likely to be misdiagnosed. Imaging examination and laboratory examination are helpful to the diagnosis of MSS. The definitive diagnosis depends on the pathologic biopsy.