Background: Total situs inversus, (also called situs transversus or oppositus) known as mirror image dextrocardia, is characterized by a heart on the right side of the midline while the liver is on the left side. Situs Inversus Totalis (SIT) is a congenital anomaly characterized by complete transposition of abdominal and thoracic organs. Patients with this anomaly usually lead a normal life. We report a fourteen-year old Nigerian who was found to have dextrocardia with situs inversus totalis while presenting for treatment for a different problem. Conclusion: Situs inversus totalis is a rare finding. This report underscores the need for routine screening of children so that such congenital anomalies can be identified and complications that follow it averted timely.