Segmental Odontomaxillary Dysplasia (SOD) is an uncommon developmental disorder of unknown etiology that causes a unilateral alteration of the maxilla associated with an abnormal growth and maturation of bone, lack of one or both premolars and delayed tooth eruption on the affected side, alteration of adjacent gingival tissue with or without facial cutaneous lesions. Radiographically is observed an irregular trabecular pattern and reduction of the maxillary sinus. There are 50 cases reported in the English-language literature. A literature review is herein presented emphasizing the clinical, radiographic, and histological features, and three additional cases of SOD affecting a 22 years-old woman, a 18 years-old man, and a 5 years-old boy, respectively are described.