Granulomatous diseases are a heterogeneous group of the diseases of different etiology, which are manifested by a variety of clinical syndromes and variants of tissue changes, by non-uniform sensitivity to therapy, and by the predominance of the common dominant histologic sign — the presence of granulomas that determine the clinical and morphological essence of each disease. Granuloma is a chronic inflammatory response, which involves macrophages and other inflammatory cells. After exposure to an antigen, T-lymphocytes, macrophages, epithelioid cells, and multinucleated giant cells are activated, resulting in the formation of granulomas. Granuloma also includes the extracellular matrix produced by fibroblasts, which can demarcate and isolate the antigen. According to etiology, granulomatous diseases are classified as infectious and non-infectious. However, recent investigations demonstrate that pathogenic microorganisms can cause granulomas in diseases previously considered non-infectious. In some cases, it is very difficult to classify a granulomatous process as infectious and non-infectious. The aim of this paper is to draw the attention of readers to the diversity of granulomatous diseases, to describe the key points of pathological and anatomical manifestations of various non-infectious diseases, as well as to determine an approach to the differential diagnosis of granulomatoses.