Histoplasmosis is endemic to the midwestern and east central states in the United States near the Mississippi and the Ohio River valleys. Ninety-nine percent of patients exposed to histoplasmosis develop only subclinical infection. Liver involvement as a part of disseminated histoplasmosis is well known; however, isolated hepatic histoplasmosis without any other stigmata of dissemination is extremely rare and the literature is limited to only two case reports. We present a rare case of isolated granulomatous hepatitis due to histoplasmosis in a 35-year-old female with dermatomyositis receiving low-dose prednisone and methotrexate. There was no evidence of fungal dissemination elsewhere. High clinical suspicion is critical for early diagnosis and treatment.