The classical manifestation of asphyxiating thoracic dystrophy comprise narrow chest, short limbs, and pelvic changes. The degree of respiratory distress varied from negligible to lethal. The development of progressive renal failure may indicate that the condition is asphyxiating thoracic dystrophy. We have experienced a case of asphyxiating thoracic dystrophy who died from chronic renal failure in a 5-month old infant. Abriefreviewofassociatedliteratureisalsopresented.