Granulomas are detected in the liver in a variety of conditions and their presence may be an important sign of the ongoing systemic disease. Although granulomas rarely cause severe liver damage at early stages of development, early identification of the underlying systemic disease may have important therapeutic and prognostic implications. The clinical presentation of granulomatous liver diseases depends on the underlying cause and its severity. The most common symptoms are fever, night sweats and weight loss. In the majority of patients minor elevations of serum aminotransferases and alkaline phosphatase may be the initial abnormality. The diagnosis is based on liver biopsy and specific testing for the underlying disease. The type of therapy depends on the aetiological factor. Infectious diseases respond well to antibacterial, antifungal, antiviral or antiparasitic drugs. Granulomas coming into existence on the base of neoplastic diseases may regress after application of chemotherapy. Glucocorticosteroids are usually used to treat the symptomatic form of granulomatous hepatitis.