Developmental disturbances involving the oral cavity affect the growth and development of a child. Tooth agenesis may be associated with a number of documented syndromes or may present as an isolated entity. The presence or absence of teeth is decided by the influence of various genes and their signaling pathways. These syndromes appear due to chromosomal defects or due to mutations in the genes responsible for organogenesis. Identification of these mutations helps understand the underlying defect and plays an important role in their treatment strategies. This is a comprehensive review of literature on syndromic and nonsyndromic forms of dental agenesis and an attempt in enlisting various syndromes associated with dental agenesis.