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. The purpose of this case report is to evaluate the dental management of patients with Steinert's disease. The patient needed dental extraction. A locore-gional paraperiosteal anesthesia was performed using bupiva-caine 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). 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. 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.