Introduction. Congenital cholesteatoma (CC) is a rare pathological condition of the middle ear characterized by keratinizing squamous epithelium located behind an intact tympanic membrane. Bilateral involvement is exceptionally uncommon and may present a significant diagnostic challenge, particularly in the absence of inflammatory symptoms. Case report. We present the case of a 19-year-old male with a 4-year history of gradually progressive bilateral hearing loss accompanied by tinnitus and otalgia, without otorrhea or prior otologic disease. Otomicroscopy revealed a pearly white mass visible through intact tympanic membranes bilaterally. Pure-tone audiometry identified severe bilateral conductive hearing loss. Multislice computed tomography of the temporal bones revealed bilateral soft-tissue masses within the middle ear cavities, along with associated ossicular erosion. Bilateral tympanomastoidectomy with ossiculoplasty was performed, and CC occupying the mesotympanum and epitympanum was confirmed intraoperatively. Postoperative follow-up with non-echo-planar diffusion-weighted magnetic resonance imaging, performed 2 years after surgery, showed no evidence of residual or recurrent disease. Conclusion. This case emphasizes the importance of considering CC in adolescents and young adults presenting with bilateral severe conductive hearing loss and intact tympanic membranes. Advanced imaging is essential for accurate diagnosis and timely surgical management, even in the absence of classic inflammatory signs.