Hypodontia is defined as the congenital deficiency of one or more teeth and is one of the most common dental anomalies in humans. Multifactorial etiology can include environmental factors as well, since a combination of environmental and genetic factors might contribute to the occurrence of dental agenesis. Patient with missing teeth; reduced masticatory ability, inarticulate pronunciation may encounter esthetics and periodontal problems. Tooth agenesis definition, etiology, genes that cause tooth agenesis and treatment of tooth agenesis is mentioned in this review. Nowadays, many genes which play role in tooth development reported to be a potential candidate gene in tooth agenesis. Functional changes seen in genes which play role in tooth agenesis are mentioned that there may be a relationship with the development of cancer and tooth agenesis may be a significant marker for cancer.