Abstract
Facioscapulohumeral muscular dystrophy (FSHD) belongs to the group of rare diseases known as muscular dystrophies. Patients with muscular dystrophies face a heightened risk of intraoperative complications, including severe hyperkalemia and acute rhabdomyolysis. This case report outlines the anesthetic approach employed for a pediatric patient diagnosed with FSHD undergoing a planned exploratory tympanotomy. To the best of our knowledge, it is the first documented case in the literature detailing pediatric general anesthesia in a patient with FSHD, with the additional use of neuromuscular blockade reversal with sugammadex.