This article is a case study discussing a case of a 40-year-old female with isolated splenic sarcoidosis found to have isolated splenomegaly with multiple hypodense splenic lesions and no other multisystem involvement. Isolated splenomegaly is very rare and an uncommon presentation of sarcoidosis. This case is clinically challenging because of the extensive differential diagnosis. The authors conclude that sarcoidosis of the spleen, although rare, should be considered in the differential diagnosis of the patients presenting with systemic symptoms and splenomegaly.