In Brief:
T1-hyperintense disc herniation can be a rare presenting sign of alkaptonuria. The T1-hyperintense disc herniation generates a narrow differential diagnosis: hemosiderosis, Wilson disease, hemochromatosis, calcification, melanin deposition, lipofuscin deposition, and alkaptonuric ochronosis. Diagnosis of alkaptonuria allows for primary and secondary prevention of ochronotic degradation of myocardium, renal parenchyma, and cartilage.