Patients with muscular dystrophies, especially those pauci-symptomatic presenting for surgery pose a complex problem for the anesthesiologist in preparing, optimizing and performing anesthesia. A myriad of complications including cardiac, respiratory, rhabdomyolysis, hyperkalemia, increased sensitivity to muscle relaxants etc., influence the anesthetic technique and recovery. Preoperative identification and appropriate choice of anesthesia technique can prevent most of the adverse events during anesthesia. We present a case of facioscapulohumeral dystrophy (FSHD) presenting for emergency appendectomy. Preoperative investigations and lung function were adequate. The patient underwent general anesthesia with propofol, cisatarcurium, and remifentanil and was maintained on total IV anesthesia for the duration of surgery. Continuous neuromuscular monitoring was carried out at two sites and the patient responded normally to intubating dose of cisatracurium and subsequent top-up doses, showing no increased sensitivity or need for dose reduction. The patient was hemodynamically stable with propofol and remifentanil infusions, with inhalational agents purposefully avoided. The patient was reversed with anticholinesterase and good train-of-four (TOF) ratio (>90%) was ensured before being shifted to recovery. The patient had a further uneventful course in the hospital.