A patient with end-stage amyotrophic lateral sclerosis (ALS) presented for Baclofen pump replacement. She underwent a left transversus abdominis plane block to anesthetize the left lower quadrant of the abdomen. No sedatives or analgesics were administered, and the procedure was successfully completed without complication. It is prudent to consider anesthetic plans that avoid complications associated with general or neuraxial anesthesia in patients with ALS. This case report demonstrates successful placement of a transversus abdominis plane block in a patient with ALS and offers a safe anesthetic technique that can be performed in other high-risk patients.