Amelogenesis imperfecta (AI), dentinogenesis imperfecta (DI), and dentin dysplasia (DD) are hereditary illnesses that affect the growth and architecture of teeth’s hard tissues (enamel and dentine). These diseases present clinical symptoms such as tooth discoloration, enamel/dentine abnormalities, and enhanced tooth sensitivity. AI is defined by teeth enamel flaws, while DI is characterized by anomalies in dentin development, leading to opalescent or yellow-brown teeth, greater translucency, and a higher risk of fractures. DD is less prevalent than AI or DI. The genetic basis of DD, DI, and AI is derived from gene alterations. Mutations in the DSPP, DMP1, COL1A1, and COL1A2 genes are linked to DI, while the DSPP, DMP1, and COL1A2 genes are the cause of DD. Family genetic history was used to detect genetic mutation and confirm diagnosis, with treatment options including endodontic therapy, restorative dentistry, and preventative treatments as in fluoride therapy and oral hygiene guidelines. Early diagnosis and effective treatment are essential for enhancing oral health and quality of life.