The management of children with defects of dentin can be challenging from both a diagnostic and treatment perspective. Obtaining the correct diagnosis is based on careful evaluation of the family history, clinical manifestations, radiographic appearance and, when indicated, appropriate laboratory tests. This chapter aims to describe the various types of conditions leading to defects in dentin with emphasis on the importance of establishing a holistic patient care plan that will encompass a stepwise approach to help achieve the short-, medium-, and long-term treatment goals of functional and aesthetic dentition.