We report a case of an adult male with X‐ALD who was genetically diagnosed with a missense mutation (c.1534G>A) of the ABCD1 gene, with normal very long chain fatty acids and no abnormalities on MRI at 2 years of onset and with brainstem lesion at 7 years of onset as the main feature. Here, ABCD1 is linked to adrenoleukodystrophy.