In primary care, universal newborn hearing screening is a key preventive service that allows early detection of hearing loss, even in infants without risk factors. Early identification and prompt referral are essential for optimal language, cognitive, and long-term outcomes. This case report outlines the evaluation and coordinated management of a term newborn diagnosed with bilateral profound hearing loss during routine screening at a family health center.A female infant was brought for a routine newborn exam. She was born at 37+5 weeks via uncomplicated vaginal delivery (3100 g) to a healthy 27-year-old mother, with no perinatal risk factors for hearing loss. Physical exam, including external ear anatomy, was normal. Otoacoustic emission (OAE) screening showed bilateral REFER on day 1 and day 7, leading to referral to a tertiary audiology center. At 3 weeks auditory brainstem response (ABR) revealed no detectable waves up to 90 dB bilaterally, consistent with profound sensorineural hearing loss. MRI/CT showed normal inner ear and auditory nerve anatomy. Genetic testing identified a homozygous GJB2 35delG mutation. The family physician coordinated multidisciplinary follow-up, parental counseling and early referral for cochlear implant evaluation and auditory-verbal therapy.This case is notable because a full-term, low-risk newborn was found to have profound bilateral hearing loss solely through routine screening, highlighting the often-hidden burden of genetic causes in clinically low-risk infants.This experience underscores the importance of adhering to the “1-3-6 rule” in primary care. Consistent screening, timely re-testing, early referral, and coordinated multidisciplinary communication are critical. Integrating stronger genetic counseling pathways within family medicine may further improve care.This case shows that profound hearing loss can occur even in the absence of risk factors, emphasizing the need for repeat screening, timely referral, and adherence to the 1-3-6 guideline in primary care to ensure early intervention and optimal outcomes.Primary care plays a key role in detecting severe hearing loss in asymptomatic newborns. Early identification enables timely intervention and improves long-term speech and language outcomes.