Children with congenital heart disease are at high risk for neurodevelopmental challenges. There is little known, however, about possible effects of changing cardiac status on neurodevelopmental profiles over time. We examined the developmental trajectory of neuropsychological skills in a child with congenital heart disease who underwent multiple procedures over the course of childhood and adolescence. Serial neuropsychological evaluation (ages 8, 10, 11, and 14), was used to determine how cognitive skills varied as a result of changing cardiac status, and showed a stable pattern of strengths, including reasoning, learning and memory, with weaknesses in attention/executive skills, processing speed, and fine motor skills. There was a steady, downward trend in some areas, such as sustained attention and flexibility, suggesting a slower trajectory of development. In other areas, including aspects of executive function, processing speed, and verbal retrieval, there was fluctuation of scores with changing cardiac status that recovered following intervention. The clinical utility of serial neuropsychological evaluation in monitoring the effects of changing cardiac status is discussed.