Sarcoidosis is an inflammatory disease with an unknown etiology. The main manifestation of sarcoidosis is due to involvement of the pulmonary system, in which it presents as a noncaseating granulomas and generalized lymphadenopathy. It is generally a multisystem disease that presents with a wide spectrum of differential diagnosis including infectious,autoimmune,neoplastic conditions of spleen. However, isolated granulomatous disease confined to spleen is rare. The approach to isolated splenic nodules in a patient with nonspecific abdominal symptoms should be focused on exclusion of malignancies and infections, and may require computed tomography, magnetic resonance imaging, and positron emission tomography–computed tomography imaging; scintigraphy; bone marrow biopsy; breast and genital examinations; and endoscopies. We report a rare case of isolated sarcoidosis of spleen which was treated laparoscopic ally.