We report on an anesthetic experience with a 74-year-old female with Isaacs' syndrome, who underwent elective surgery for open rotator cuff repair. Isaacs' syndrome is a rare peripheral motor neuron disorder with clinical manifestations such as involuntary muscle twitching, cramps, mild weakness and increased sweating. To avoid prolonged neuromuscular blockade, the patient was observed with neuromuscular monitoring during total intravenous anesthesia with propofol, remifentanil, and atracurium. No adverse events were observed during the anesthetic management, and the patient recovered smoothly from the neuromuscular blockade. We describe the clinical characteristics of Isaacs' syndrome and its specific considerations in anesthesia.