Sarcoidosis is a granulomatous disease with multiple system involvement. It is characterized by the presence of non-caseating granulomas in the involved organs. The most commonly affected organ in sarcoidosis is the pulmonary interstitium. However, extra-pulmonary involvement can be manifested in almost any other organ system. Less commonly, sarcoidosis can manifest with massive splenomegaly. As extensive differential diagnoses, such as hematological malignancies, primary splenic or metastatic tumors, infiltrative disorders, and inflammatory disorders are considered, diagnosing sarcoidosis with the presentation of massive splenomegaly is clinically challenging. Here, we discuss the case of a 56-year-old female with splenic sarcoidosis and skin manifestation.