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Background: Osteopetrosis (OP) is a rare disease of the skeletal system that can be associated with complications such as bone fracture, nerve dysfunction and deafness due to increased bone density and reduced bone quality. In this regard and due to the challenge that it can cause for cochlear implantation (CI), in this study we aimed to report CI conducted on two patients with OP in Iran. The Case: Patients were two women diagnosed with OP and bilateral sensorineural hearing loss (SNHL) who underwent CI in the right ear. Preoperative PTA showed a hearing threshold decrease of more than 100 dB in both patients. A standard cochleostomy was performed in one patient and endoscopic surgery in the other pati­ent through the external ear canal. One month after surgery, the hearing threshold improved by 60−90 dB in both patients. No facial nerve palsy or implant extrusion/migration was observed after surgery. Conclusion: Although technically challenging, CI seems to be a safe and effective method to improve the SNHL in patients with OP. The path for electrode insertion should be tailored to meet the conditions and anatomy of patients.