Abstract. Objectives: Mercury is a dangerous element, particularly for children, since it can easily be found in the environment. In this study, for the first time in literature, it was aimed to evaluate the levels of neuron-specific enolase (NSE) and S100B as neurotoxicity markers in children with mercury intoxication. Materials and methods: A total of 85 subjects with acute mercury intoxication (53 females and 32 males) were included in the study. The control group consisted of 82 healthy children aged between 6 and 18 years (50 females and 32 males). Patients with a urine mercury level > 15 mg/L were considered to have mercury poisoning. ELISA assays were used to analyze S100B and NSE protein levels prior to treatment. Results: NSE and S100B levels were high in the patient group when compared to the control group (p < 0.05). No difference was detected in NSE, S100B, and mercury levels with respect to gender (p > 0.05). The patients were divided into two age groups of 6 – 12 years and 13 – 18 years, and no significant difference was detected in age-dependent blood levels of mercury, NSE and S100B (p > 0.05). No significant differences were found in NSE and S100B levels between the patients with and without neurological findings (p > 0.05). Conclusion: NSE and S100B protein levels, which are biochemical indicators of neurotoxicity, are elevated in acute exposure to mercury.