Objective: Subclavian Steal Syndrome (SSS) is a disorder characterized by an occlusion or stenosis of the subclavian artery causing retrograde blood flow in the ipsilateral vertebral artery, typically diagnosed in men over the age of 55. SSS can produce neurologic symptoms, and case studies in adults report temporal and spatial disorientation, cognitive deterioration, and attention and memory deficits. The disorder is rarely diagnosed in childhood, and no previous studies following neurocognitive symptoms were found. Method: This 13-year-old African-American male had a vascular ring anomaly diagnosed and repaired at age 9 months, and was doing well until age 12 when he began experiencing multiple episodes of syncope. He was diagnosed with SSS and underwent surgical repair and graft. He has continued to experience syncope secondary to orthostatic hypotension, and has profound hearing loss in the right ear and myopia. Neuropsychological concerns included decline in academic performance and poor attention. Results: Results of neuropsychological testing showed borderline Full Scale IQ (72), despite previous high academic achievement, and achievement scores ranging from low average to average. This is believed to represent a decline in cognitive abilities. Memory was average to low average. Attention, executive functioning, motor coordination, and visual-perception were impaired, and visual field examination revealed a left superior quadrantenopia. Conclusion: Borderline impaired FSIQ (representing possible cognitive deterioration) and deficits in attention are consistent with results of adult studies. Although rarely seen in childhood, neurocognitive symptoms are reported in case studies of SSS but without neuropsychological follow-up. This study offers insight into the pediatric presentation of SSS.