Patients with congenital conductive hearing loss present a surgical challenge. Surgeons must be aware of the potential altered anatomy, in particular the potential risks to the facial nerve and inner ear structures; however, meticulous attention to detail in the preoperative evaluation and in the operating room can minimize these risks. If surgery poses too high a risk to these structures, the chances of success are low, or there is a contraindication to surgery, traditional air-conduction hearing aids or bone-anchored hearing aids should be considered.