Objective: Krabbe disease (globoid cell leukodystrophy) is a rare, autosomal recessive neurodegenerative disease. It is characterized by progressive demyelination in the central and peripheral nervous systems, resulting in deterioration of cognitive and motor skills (Suzuki, 2003). Although the evidence for cognitive impairment associated with Krabbe disease is robust, profiles of neuropsychological functioning have not been well established. Method: Neuropsychological case study of a five-year-old, right-handed, Caucasian male with late-infantile onset (age three years) Krabbe disease and a history of developmental and language delay. Currently, behavioral concerns and decline in cognitive functioning are affecting his academic performance. Neuropsychological findings were compared to results of psychological, speech, and language evaluations conducted one-year prior. Results: Current overall cognitive functioning ranged from borderline to average. Neuropsychological deficits included weak memory, ongoing processing, and visual perceptual abilities. Pronounced attention problems impacted upon performances across domains and fine motor skills were consistently compromised. Mild decline in cognitive functioning since evaluations one-year prior was evident. Behavior rating scales indicated attention and withdrawal to be areas of clinical significance. Conclusion: Research outcomes on neuropsychological profiles associated with Krabbe disease have been very limited. Neuropsychological findings from this case were consistent with pathogenesis and neuroimaging literature on the central role of white matter degradation in Krabbe disease (Poretti et al., 2014). These initial results encourage further investigation into the trajectory of cognitive functioning in patients with Krabbe disease, especially as recent advances in therapeutic options show promise for this population.