This case report presents the endodontic treatment of a maxillary right lateral incisor with a perforating internal resorption in a 50-year-old woman. Radiographically, internal resorption appears as a fairly uniform, radiolucent enlargement of the pulp canal and distortion of the original root canal outline. The use of cone beam computed tomography can help the clinician in making a confirmatory diagnosis and determining the treatment plan before undertaking the actual treatment. After cleaning the root canal space and the resorptive defect by mechanic instrumentation, irrigation, and interim calcium hydroxide dressing, the apical third canal was filled with a gutta-percha point by lateral condensation. The resorptive defect was filled with mineral trioxide aggregate. Follow-up radiographs at 3 years showed adequate repair of the resorption, and the tooth remained asymptomatic. Keywords: cone beam computed tomography, mineral trioxide aggregate, perforating internal resorption