Objective:This paper presents a rare case of macrodontia together with double form of Dens invaginatus (DI). Methods:In this case macrodontia was together with double form of Dens invaginatus (DI) in the right permanent maxillary central incisor, single form of DI in the left permanent maxillary central incisor. The condition was associated with oligodontia and persisted primary mandibular incisors. However no developmental syndrome was identified. The maxillary left central incisor required no treatment and a fissure sealent was applied for caries prevention. Root canal treatment was indicated for the maxillary right central incisor that had an open apex with irregular root canal system. In addition, right central incisor was formed smaller and left central incisor was formed larger then their original size with a composite resin. As the response of the electrical pulp testing was normal for the retined mandibular primary central incisors and aesthetic appearence was satisfactory for the patient, no treatment was applied for those teeth. Results:Conclusion: After 18 months clinical and radiographical results demonstrated the effectiveness of treatment modalities.

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