On the occasion of the death of Annette Karmiloff-Smith, this article presents a review of the work and research career of this author, with special emphasis on her contributions to contemporary developmental psychology, neuropsychology and cognitive science. The central elements of her theoretical proposal and the main evidence that give empirical support to her perspective on typical and atypical cognitive development are presented. Additionally, the impact of her ideas on current discussions in psychology and cognitive science is traced, and some lines of work that the author herself proposed for future research are explained. In general, Karmiloff-Smith's work is relevant in current research in psychology due to the specific but common lines of research she addressed between psychology, neuroscience and genetics, posing a collaborative and methodological background between these areas that will continue to offer evidence on the factors that characterize the variability of human neurocognitive development.