Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon hereditary arrhythmogenic syndrome, with an estimated prevalence of ∼0.1/1000. In this disease, myocardial calcium receptors are, more than usual, sensitive to catecholamine release, leading to severe tachyarrhythmias after emotional or physical stress. To the best of our knowledge, only a few cases of general anesthesia (GA) in patients with CPVT have been reported in literature, and none for potentially arrhythmogenic procedures like laser lead extraction. We describe the case of a 17-year-old male patient with CPVT, undergoing GA, for laser lead extraction of an old implantable cardioverter-defibrillator. The procedure, conducted in totally intravenous anesthesia, was uneventful, and just a few sporadic ventricular extrabeats owing to mechanical manipulation of the old wire were reported. The patient was extubated in the operation theater and discharged the following day. In the discussion, the case is analyzed, the main issues highlighted, and different anesthetic strategies considered. In conclusion, laser lead extraction in patients affected by CPVT can be performed safely under GA, provided that patients are cautiously treated and assessed during the whole perioperative period.