The skull base is a critical structure in the craniofacial region, supporting the brain and vital facial structures in addition to serving as a passageway for important structures entering and exiting the cranial cavity. This paper will review and highlight some of the embryology, developmental anatomy, including ossification, and related abnormalities of the anterior, central and posterior skull base using illustrative cases and tables. Pathologies such as dermoids/epidermoids, cephaloceles, nasal gliomas, glioneuronal heterotopias, various notochordal remnants, persistent craniopharyngeal canal, teratomas, platybasia, basilar invagination, clival anomalies and Chiari malformations will be discussed. Developmental pearls and pitfalls will also be highlighted.