Patients with amyotrophic lateral sclerosis (ALS) who undergo lower extremity joint arthroplasty are rarely encountered. Patients with ALS are at an increased risk for perioperative anesthetic complications. Anesthetic techniques, regional or general, present different risks to patients with ALS. The historical concern of worsening pre-existing neurologic symptoms with regional anesthesia is being re-examined in light of emerging evidence supporting its use in patients with ALS. Here, we present the successful perioperative management of a patient with severe bulbar ALS undergoing total knee arthroplasty. Despite his advanced bulbar symptoms, he was independently ambulatory with severe knee pain related to osteoarthritis. During multidisciplinary planning with the patient and his wife, it became clear that his primary perioperative concern was avoiding intubation, prolonged ventilation, and tracheostomy placement. With this in mind, we planned for a neuraxial anesthetic without intraoperative sedation, a postoperative adductor canal peripheral nerve block, and multimodal non-opioid analgesia. There were no perioperative complications. At the six-week follow-up, he experienced improved ambulation and showed no signs of worsened ALS symptoms.