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Background and Objectives: Steinert disease is the most common muscular dystrophy of the adult. Due to its multisystem characteristic, the perioperative management of these patients is a challenge to the anesthesiologist. The aim of this report was to present a case of hemorrhoidectomy in a patient with muscular dystrophy and to discuss the several anesthetic implications involved., Case Report: A man patient, 58 years old, with Steinert disease, who underwent hemorrhoidectomy. Subaracnoid block with hyperbaric bupivacaine (saddle block with puncture at L3-L4 with 0.5% bupivacaine [5 mg]) associated with sedation with propofol (1 microg.mL-1 target using a target-controlled infusion pump). Dypirone (1.5 g) and local infiltration with 0.5% ropivacaine (150 mg) were used for the postoperative analgesia. Intraoperatively, the patient developed myotonic crisis (10 minutes after being placed on the litothomy position) that was controlled by sedation (the target concentration was increased to 1.5 microg.mL-1 and given a bolus of 40 mg). The patient remained stable and was discharged the following day., Conclusions: The knowledge about the disease and the proper anesthetic planning are extremely important when managing patients with Steinert disease.