Different studies have demonstrated changes in chitotriosidase (ChT) activity and concentrations in multiple diseases. However, changes in ChT activity and concentrations have not been concurrently evaluated in patients with Familial Mediterranean Fever (FMF). In this study, we analyzed the changes in serum ChT activity and concentrations in patients with FMF. The study included a total of 80 patients with FMF and 80 healthy controls. ChT enzyme activity and concentrations were measured and then compared between the groups. ChT activity was measured by using fluorometric ELISA and ChT concentrations were measured by using colorimetric ELISA methods. The median ChT activity was 10.00 (6.00-15.00) nmol/mL/hr in the patients and 14.00 (6.25-20.75) nmol/mL/hr in the controls. There was a statistically significant difference in the ChT activity between the controls and patients (P= 0.027). The median ChT concentrations were 65.40 (46.20-84.92) pg/mL and 125.00 (75.72-143.95) pg/mL in the patients and controls, respectively (P< 0.001), which were expressed as median percentiles (25th-75th). Additionally, we found no correlation between C-reactive protein and ChT activity (P= 0.978, r= 0.003) and concentrations (P= 0.446, r= -0.87). Serum ChT enzyme activity and concentrations may not be considered as a biomarker in FMF patients taking colchicine. New studies are needed to evaluate the changes of enzyme activity and concentration in colchicine-negative patients.