Mesiodens are the most common supernumerary teeth and are detected incidentally during routine radiographic examination, so late diagnosis complications are very common. The dentist must make a timely diagnosis and thus avoid clinical complications. Despite advances in knowledge of dental morphogenesis and differentiation, the etiology of mesiodens remains unclear. Therefore, several theories have been postulated to explain how and why they develop. It was described in the literature that heredity could play an important role in the appearance of supernumerary teeth, with a higher rate of appearance in relatives of those affected. This article reports three cases, a mother and two children, who present mesiodens, which shows that supernumerary teeth may involve a genetic factor. In addition, a literature review was carried out to assess the importance of the genetic factor as a possible cause of mesiodens. The relevance and implications of timely diagnosis in clinical practice to avoid manifestations of clinical complications are discussed. Therefore, the identification of the genetic risk factors responsible for the formation of supernumerary teeth is essential for developing a screening tool to determine an individual’s genetic risk.