Hypertrophic olivary degeneration (HOD) is a rare condition characterized by degeneration and hypertrophy of the inferior olivary nucleus (ION) in the brainstem. This case report presents a 39-year-old woman with HOD undergoing left total shoulder arthroplasty. Despite long-standing concerns about regional anesthesia in patients with central nervous system disorders, an interscalene catheter was placed using ultrasound and nerve stimulation guidance. The patient experienced effective analgesia without new neurologic deficits. This report highlights the safe use of regional anesthesia in patients with HOD and underscores the need for individualized anesthetic management and further research to establish clear guidelines.