The patients with complicated congenital heart diseases are reaching adulthood with advances in corrective surgeries and medical management. Impact of anesthetic agents on complex cardiac and extra cardiac anomalies and presence of previous palliative procedures can be a challenge for the anesthesiologist perioperatively, while these patients present for cardiac/noncardiac surgeries. We report the perioperative management of a patient with ventricular septal defect, dextrocardia, pulmonary hypertension, and situs inversus who underwent a successful hernioplasty and hydrocelectomy with a combined spinal epidural anesthesia. This discussion relates to the anesthetic management in such conditions with a special reference to Kartagener's syndrome.