Cystic fibrosis (CF) was considered to be non-existent in Indian subcontinent. 
Reports in last one decade have suggested that cystic fibrosis occurs in India 
but its precise magnitude is not known. Studies on migrant Indian population in 
United States and United Kingdom estimate frequency of CF as 1:10,000 to 
1:40,000. The clinical features are similar to that reported in Caucasian 
population. CF in Indian children is usually diagnosed late and in advanced 
stage. Children are more malnourished and may have clinically evident deficiency 
of fat soluble vitamins. The frequency of clubbing, colonization with 
Pseudomonas, and laboratory evidence of pseudo-Bartter syndrome is relatively 
more at the time of diagnosis. Diagnostic facilities in form of sweat chloride 
estimation and genetic studies are not available readily. Mutation profile is 
different. The frequency of common mutation F508del in Indian children is 
between 19% and 34%. Other mutations are heterogeneous. Management of CF in 
India is difficult due to less number of trained manpower, limited availability, 
and high cost of pharmacologic agents. The determinants of early death include: 
severe malnutrition and colonization with Pseudomonas at the time of diagnosis, 
more than four episodes of lower respiratory infection per year and age of onset 
of symptoms before 2 months of age. To conclude, CF does occur in India; 
however, precise magnitude of problem is not known. There is need to create 
awareness amongst pediatricians, developing diagnostic facilities, and 
management protocols based on locally available resources.