A 28-year-old male with dentatorubral pallidoluysian atrophy (DRPLA) was scheduled for evulsion of bone marrow nail after tibia fracture. He was diagnosed as having DRPLA at the age of 12. Although he was receiving anticonvulsants, regular seizures had occurred occasionally and his symptoms were exacerbating. General anesthesia was induced and maintained with intravenous propofol supplemented with fentanyl, nitrous oxide, and oxygen. The operation was performed uneventfully. After the surgery, his breathing was depressed and naloxone was administered to reverse the depression. Thereafter, generalized tonic seizure associated with involuntary movement of the left arm occurred. These symptoms ceased soon after rapid injection of diazepam. Inhaled and intravenous anesthetics have been alleged to have both proconvulsant and anticonvulsant activities in humans. In the present patient with DRPLA, propofol, fentanyl, nitrous oxide, naloxone, and neostigmine administered might be factors, which could have lowered the threshold for seizure activity. Further, patients with DRPLA might have decreased GABA in the basal ganglia, and anesthetics would affect GABA receptors. Thus we should be aware of occurrence of convulsion in DRPLA patients during and after anesthesia.