Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology, which is characterized by a variable clinical presentation and course. The diagnosis of this disease is usually supported by the three elements: compatible clinical and radiologic findings, tissue biopsy specimen that reveals noncaseating epithelioid granulomas and the absence of known granulomagenic agent. During the last years the new diagnostic methods have been discovered, but serum markers of the sarcoidosis are still under studying. Among the potential markers of sarcoidosis, a recently proposed indicator is chitotriosidase, a chitinase produced by chronically activated macrophages. The review of the literature showed that chitotriosidase activity is only a surrogate biomarker to confirm diagnosis, but is a useful marker for disease activity monitoring and prognosis. The correlation with the radiological stages of disease suggest that determination of chitotriosidase activity could decrease the number of X-ray examination.