Objective: Adrenoleukodystrophy (ALD) is a rare X-linked inherited metabolic disease associated with an accumulation of very long chain fatty acids in the adrenal glands and the central and peripheral nervous systems. The most frequent phenotype is adrenomyeloneuropathy (AMN). AMN is characterized by progressive axonal degeneration affecting the spinal cord and peripheral nerves. While the occurrence of potential neuropsychiatric symptoms in AMN has been well-documented, AMN had long been considered a mild ALD phenotype with only infrequent cerebral involvement. However, recent studies suggest development of cerebral demyelination may occur in anywhere from 20-63% of AMN patients. Further, brain magnetic resonance imaging (MRI) in AMN patients with neurological symptoms suggestive of cerebral involvement can initially appear normal. Descriptions of the neuropsychological deficits seen in adult ALD and AMN phenotypes are lacking and have been accompanied by unknown or positive neuroimaging findings. We present the psychological and neuropsychological profile of an adult with AMN and normal MRI findings. Method: We describe a 48-year-old man affected by AMN whose symptoms appeared 24 years ago in the form of spastic paraparesis and amyotrophy, followed shortly by psychiatric symptoms of hypomanic episodes and gradual, subtle changes in personality. Veteran underwent MRI, neuropsychological, and psychological assessment of functioning. Results: The neuropsychological findings configure a picture of impaired executive functioning and reasoning abilities in the presence of normal MRI. Conclusion: Neuropsychological examination may be useful in identifying cognitive decline in AMN patients prior to the onset of positive MRI findings.