Background: Myotonic dystrophy type I (DM1) is a genetic autosomal dominant disorder; malignant hyperthermia is a possible complication. It may occur following administration of some halogenated general anesthetics, muscle relaxants, or surgical stress Aim: The purpose of this case report is to evaluate the dental management of patients with Steinert's disease. Case report: The patient needed dental extraction. A locoregional paraperiosteal anesthesia was performed using bupivacaine without vasoconstrictor and sedation with nitrous oxide. The syndesmotomy of the elements 3.1, 4.1, and 4.2 was executed. The elements were dislocated through a straight lever and avulsed with an appropriate clamp. The socket was courted, washing with saline solution, inserting a fibrin sponge, and applying sutures (silk 3-0). Conclusion: Dental treatment of the patient with Steinert's dystrophy must be carried out under a hospital environment and the use of local anesthetic without vasoconstrictor and with use of nitrous oxide; anxiolysis is recommended. Clinical significance: This case report describes the precautions to perform oral surgery in patients with Steinert's disease and emphasizes the role of anxiolysis to avoid episodes of malignant hyperthermia.