Congenital defects of the scalp and skull present a challenge for care providers because of a combination of their rarity and the magnitude of potential morbidity. Recent advancements in autogenous and alloplastic cranioplasty and scalp reconstruction techniques argue for a comprehensive consideration of this problem. This article (1) reviews the causes of congenital scalp and calvarial defects; (2) proposes a classification system based on defect type, similar to the tumor-node-metastasis classification, in that defect location, defect size, and extent of neuromeningeal involvement are the critical variables; and (3) presents algorithms for care based on the defect classification. A set of management principles on which treatment plans can be based for these unique problems is provided.