To highlight a case of cochlear implantation in the setting of ipsilateral petrous apex chondrosarcoma. A patient with bilateral progressive hearing loss was incidentally found to have a destructive right petrous apex lesion on computed tomography before cochlear implantation. The patient had no associated symptoms and a magnetic resonance imaging scan was obtained, narrowing the differential diagnosis. A middle cranial fossa approach was performed for synchronous biopsy of the lesion and cochlear implantation. Frozen sections revealed a low-grade chondroid lesion, and a Med-El Combi 40+ cochlear implant with a split electrode array was inserted via the middle fossa. Final pathologic examination revealed a Grade I chondrosarcoma. The patient suffered no complications postoperatively and was followed-up over 5 years with serial computed tomographic scans and clinical examinations. No additional treatment was administered. Eighteen months postoperatively, the patient experienced episodic vertigo. There were no new findings on computed tomography, and the vertigo improved with a low-salt diet. Otherwise, the patient had excellent hearing results, and the lesion has not progressed under observation. The implications of observing low-grade chondrosarcomas in well-selected patients and the unique aspect of cochlear implantation on the affected side are discussed.