Background: Dextrocardia is a congenital abnormal position of the heart in which the main part of the heart is in the right chest and the long axis of the heart points to the lower right, cases with combination of dextrocardia and sick sinus syndrome are rare.Case presentation: A 65-year-old female patient was admitted with palpitations and dizziness for 1 week. Mirror-image Dextrocardia and sick sinus syndrome was diagnosed by electrocardiogram (ECG), echocardiography, Holter monitoring, and X-ray. The rarity of the case and the specificity of anatomy brought great challenges to our interventional treatments. Finally, we successfully implanted a DDD pacemaker into the patient.Conclusion: For dextrocardia, using active fixation leads in both atrial and ventricular leads is easier to find the position with optimal sensing and pacing threshold, which also have a lower and more stable pacing threshold and can reduce the incidence of falling off at the same time. During operation, combine the multiple positions under fluoroscopy to confirm the leads position, which can improve the success rate of implantation.