Abstract
Isomerism or Heterotaxy syndromes are rare multifaceted congenital anomalies with multi-system involvement. Grouped under the broad category of Situs Ambiguous defects, these often pose diagnostic difficulties due to their varied and confusing anatomy. Since patients rarely survive into adulthood due to cardiovascular complications, the etiology and natural history of such conditions are not fully understood. Imaging provides the most accurate non invasive method for diagnosis and thereby, prognosis in such cases. We present a case of right sided Isomerism with complex cardiac anomalies in a 17 year old adolescent, who presented with dysphagia as one of the main complaints. Multi modality imaging demonstrated the intricate abnormalities in vital systems.