The development of the middle and inner ear highlights the intricacy of embryology. As early as 3 weeks after fertilization, the inner ear begins taking form. This process, along with development of the middle ear, continues throughout gestation. At birth, the middle ear, inner ear, and associated structures are almost adult size. An understanding of the embryologic development of the ear serves as a foundation for evaluating and managing congenital malformations of these structures. The focus of this article is the normal, abnormal, and arrested development of the middle and inner ear, with a clinical emphasis on malformed middle and inner ear structures and a discussion of associated syndromes.