Introduction: Asphyxiating thoracic dysplasia (Jeune syndrome) is a rare hereditary skeletal disorder characterized by a constricted thoracic cage leading to life-threatening respiratory compromise. The condition carries a high neonatal mortality , with early diagnosis posing significant clinical and ethical challenges. Case presentation: We describe a preterm neonate born to non-consanguineous healthy parents following an antenatal course complicated by polyhydramnios. An anomaly scan raised suspicion of thoracic dysplasia. Delivery was performed via emergency lower segment caesarean section at 34 weeks of gestation. The infant weighed 2.6 kg with a length of 38 cm. Postnatal examination revealed facial dysmorphism, rhizomelic limb shortening, a markedly narrow thoracic cage, and an associated omphalocele — features consistent with asphyxiating thoracic dysplasia. Severe respiratory distress was evident from birth due to restricted thoracic expansion, necessitating ventilatory support. Despite intensive care measures, respiratory failure remained refractory , and the infant succumbed within 48 hours of life. Conclusion: This case highlights the lethal impact of thoracic restriction in Jeune syndrome and underscores the vital role of antenatal suspicion in guiding parental counselling and perinatal preparedness. Early recognition transforms uncertainty into informed decisionmaking, enabling clinicians and families to anticipate the profound respiratory challenges and navigate care with clarity in the face of a condition where survival is often limited.