Duchenne muscular dystrophy is a hereditary disorder characterized by progressive muscle weakness and contracture, and special care during anesthesia is needed in these patients. Because inhalational anesthetics and succinylcholine can cause fatal results, intravenous anesthetics are commonly used. However, monitorings for the pediatric population are not otherwise specified. We report our experience of a 6 year-old boy that underwent muscle biopsy suspicious of muscle dystrophy under general anesthesia. The patient received midazolam, fentanyl, propofol and a small dose of rocuronium. He was monitored with bispectral index (BIS), acceleromyography (TOF). At the end of surgery, recovery of TOF ratio to 90% was evaluated, followed by injection of pyridostigmine and glycopyrrolate. When reversal of neuromuscular block was confirmed quantitatively and clinically, the patient was extubated and he experienced no complication.