Distigmine sometimes causes severe adverse events, and the serum butyrylcholinesterase (BChE) level is reduced by distigmine. However, limited data are available on the association between serum BChE level and the risk of distigmineinduced adverse events. To clarify the association between the risk of distigmine-induced adverse events and serum BChE level, we retrospectively reviewed 371 patients with 2199 measurements of serum BChE levels at three hospitals, of whom 24 (6.5%) had adverse events. Multivariate logistic regression analysis was performed to identify risk factors associated with adverse events of distigmine. The risk of adverse events was associated with distigmine dose > 0.15 mg/ kg/d [odds ratio (OR) 3.55, 95% conˆdence interval (CI) 1.07 11.70, p = 0.04], serum BChE level 80 112 U/L (OR 3.13, 95% CI 1.03 9.52, p = 0.04), and serum BChE level < 80 U/L (OR 13.70, 95% CI 5.14 36.50, p < 0.01). Serum BChE might be a useful biomarker for estimating the risk of distigmine-induced adverse events. The risk of adverse events might be decreased by closely monitoring serum BChE and assessing distigmine dose in patients with high risk factors.