Congenital Myotonic Dystrophy [CMD] is an autosomal dominant, trinucleotide repetition, and maternally transmitted disorder involving multiple systems. CMD is a rare genetic disorder with pediatric mortality rates up to 40%. It has a characteristic biphasic course with a neonatal phase of immobility, hypotonia, respiratory insufficiency, and feeding difficulties that often improve over first year of life and manifest again in later years as adult onset myotonia, muscle atrophy, and cataracts. Prognosis depends on the need for prolonged ventilation and associated complications. Respiratory failure in Congenital Myotonic Dystrophy is a complex process that, in part, resembles respiratory failure in other muscular/neuromuscular pathologies, where respiratory muscle weakness is the key factor. Other factors that play a role in respiratory failure in patients with myotonic dystrophy include central respiratory drive dysfunction, sleep apnea, upper airway dysfunction, and decreased chest wall compliance. The most fatal complications arise from respiratory difficulties due to profound weakness of the respiratory muscles and diaphragm which is exacerbated by secretion burden. There have been both direct and indirect studies that support the use of negative pressure ventilation for CMD patients. Recent cuirass devices offer 3 modes, continuous negative pressure, control, and secretion clearance that can be utilized for patients benefits. Negative pressure ventilation [NPV] has been used for the management of respiratory failure and secretion clearance in many neuromuscular disorders including CMD. Studies suggest that NPV can improve respiratory muscle weakness by decreasing the diaphragmatic atrophy seen traditionally with positive pressure ventilation. Biphasic cuirass ventilation may be advantageous in the management of respiratory complications of congenital myotonic dystrophy by aiding weakened respiratory muscles, improving lung expansion, and assisting in airway clearance. The uses of NPV by means of biphasic cuirass devices [BCD] are it is easy to use, and may avoid intubation and prolonged mechanical ventilation, act as a bridge to extubation, and provide airway secretion clearance.