Introduction: Congenital myopathies are rare inherited muscle disorders that often present in the neonatal period with hypotonia and respiratory compromise. Early recognition is crucial but challenging due to nonspecific clinical features. Case presentation: A term female neonate was born via normal vaginal delivery to a 22-yearold primigravida mother with thirdtrimester polyhydramnios. Immediately after birth, the infant presented with mild respiratory distress and generalized hypotonia. APGAR scores were 6 and 10 at 1 and 5 minutes respectively . On day two, the neonate developed convulsions. Persistent respiratory distress worsened on day 16, requiring intubation and mechanical ventilation. Multiple extubating attempts over following weeks failed, with consistent recurrence of respiratory distress. Standard investigations including chest radiography , echocardiography , and metabolic panels were unremarkable. Given persistent ventilator dependence, ENT consultation was obtained and muscle biopsy performed. Tracheostomy was done at day 33 for long-term ventilatory support. Muscle biopsy revealed histopathological features consistent with congenital myopathy , including fiber size variation, central nuclei, and structural abnormalities. Post-tracheostomy , the infant showed improved respiratory status with satisfactory spontaneous breathing. The child was discharged home on tracheostomy care without mechanical ventilation. Tragically , the patient died at home on day 5 following discharge. Conclusion: This case highlights the diagnostic challenges of congenital myopathy inneonates with respiratory failure. Muscle biopsy provided crucial diagnostic confirmation, enabling appropriate prognostic counseling and palliative care planning for the family . Early consideration of neuromuscular disorders is essential in neonates with unexplained hypotonia and ventilator dependence.