This case report presents the management of a case of rarely seen perforating internal replacement resorption using calcium hydroxide (CH) medication and mineral trioxide aggregate (MTA) root canal obturation. A maxillary central incisor of a 20-year-old female was showing a sinus track on the mucosa. Radiographically, an irregularly shaped and perforating internal resorption area was seen at the middle third of the root canal. Appearances of the bone-like fuzzy material were observed inside the resorption site. The apical part of the canal was obliterated. Following 3 months of CH medication, the canal and the perforation were obturated with MTA filling. At 6-year follow-up, the tooth was clinically asymptomatic and showing radiographical appearances of the hard tissue repair, resembling a barrier and periodontal membrane healing around MTA at the perforation site.