Congenital hypothyroidism (CH) is one of the most preventable causes of intellectual disability in the world. Screening programs have led to earlier detection of CH, and children with adequate thyroid supplementation can have minor long-term differences in overall neuropsychological testing compared to baseline. However up to one- fourth of children born with CH suffer from hearing loss even with early and adequate thyroid hormone supplementation. We report a rare case of a patient with hearing loss attributed to congenital hypothyroidism who had complete recovery of hearing after early thyroid hormone replacement.