Although respiratory distress syndrome (RDS) is a leading cause of neonatal morbidity and mortality in the west, there are few data on the prevalence and spectrum of RDS from developing countries. Available evidence suggests that the disorder may be less common than the overall 1% incidence reported from developed countries. In a prospective study of the prevalence of RDS in a consecutive 10134 births at the Aga Khan University Hospital, we documented the disorder in 127 (1.2% births), with a prevalence of 12.8% among low birthweight infants. The overall mortality for this group was 39%, with the highest mortality rate (68%) among newborn infants ≤ 1000 g birthweight. Our data from a large and relatively well-nourished hospital-born population in Karachi suggest that RDS is a significant cause of morbidity and mortality in preterm infants with a similar prevalence rate to western figures.