Odontoma-dysphagia syndrome is a malformation syndrome, characterized by odontomas (undifferentiated mass of the esophagus) and severe dysphagia. Odontomas are benign tumors of the upper and lower jaw and, among the odontogenic tumors, odontomas are most highly differentiated and include tissue giving rise to teeth: enamel, dentin, cementum, and pulp tissue (Nelson and Thompson 2010). Localized odontoma has been described frequently in the literature (Nelson and Thompson 2010). But the occurrence of multiple odontogenic tumors in man has hitherto been rarely observed (Bordini et al. 2008). However, the first case report of an odontoma syndrome, in 1967, described a girl suffering from multiple odontoma of the upper and lower jaw. The tumors were excised for the first time 18 days after birth. In the following 5 years, odontomas were excised twice. At the age of 6 years, the girl died after an operation for stenosis of the esophagus. Autopsy confirmed the esophageal stenosis and additionally revealed aortic stenosis, bronchiectases, chronical pyelonephritis, and interstitial hepatic sclerosis (Bader 1967). In 1973, Schmidseder and Hausamen described for the first time the odontoma dysphagia syndrome in a report on the present family (Schmidseder and Hausamen 1973). These authors observed multiple odontogenic tumors in combination with other malformations in a father and in his three children, which lead to identification of this disease as a heritable syndrome. Occasionally, cardiac (stenosis of the intrathoracic descendent aorta and interstitial myocarditis), renal (pyelonephritis), and hepatic (hepatic sclerosis) involvement has been described. Hypertrophy and dysmotility of the esophageal smooth muscles is suggested to have causative role for dysphagia (Bordini et al. 2008). Less than ten cases have been reported so far in the medical literature with odontoma-dysphagia syndrome. Both males and females may be affected, and individuals of all racial and ethnic groups may be affected (Fig. 1).