Sarcoidosis is a chronic granulomatous inflammation. The clinical spectrum in childhood is heterogeneous. Angiotensin converting enzyme activity is used as a marker for disease activity. Human chitotriosidase is produced in macrophages. In this study, serum chitotriosidase levels were significantly higher in active sarcoidosis than in inactive disease or healthy controls. Serum chitotriosidase concentrations may be a useful marker for monitoring disease activity in sarcoidosis.