Molarization and premolarization of anterior teeth have never been reported before. Double dens invaginatus is an extremely rare condition. We describe an affected female who had developmental delay and congenital progressive sensorineural hearing loss. Double dens invaginatus of molarized maxillary central incisors and premolarized maxillary lateral incisors were present. In addition, multituberculated mandibular incisors, canines, and first premolar were observed. Histologically, tooth structure was unremarkable. Family history of dental abnormalities and hearing loss was denied. Clinical and radiographic examinations were those performed when the patient was 7 and 13 years old. The basic defect is postulated to be of mesenchymal origin.