The heterotaxic syndromes may manifest as complex disorders with multiple anomalies, which have been described in the polysplenia/asplenia syndromes, or as a single isolated anomaly. Clinically insignificant anomalies may be incidentally discovered and should be recognized. Early diagnosis in the prenatal and neonatal periods allows for early intervention and correction, particularly of cardiac anomalies. Chest radiography, sonography, CT, radionuclide imaging, and MR imaging are useful diagnostic tools in evaluating these patients.