OBJECTIVE To describe the clinical features and imaging characteristics of nodular splenic sarcoidosis. METHODS We describe a patient with splenic sarcoidosis and review the related medical literature, the etiology, symptomatology, pathology, diagnosis, differential diagnosis, management and prognosis of splenic sarcoidosis. RESULTS The etiology of this rare disease remains unknown. Symptoms are scanty and usually mild; computed tomography usually reveals splenomegaly or the presence of multiple nodules, confusing with metastatic tumor in spleen. On histopathologic examination, sarcoid produces noncaseating granulomas. Sarcoid is typically treated only when symptomatic. Oral corticosteroids is the most important method of treatment in patients with progressive loss of organ functions. Prognosis has closed relationship with early clinical manifestation. CONCLUSIONS Splenic sarcoidosis is rare and often misdiagnosis as other diseases.