Cystic fibrosis is the most common life threatening, inherited disease of the Caucasians. Ever since the gene for this disease was delineated in 1989, there are about 900 mutations discovered so far. This disease is being reported with increasing frequency from North India now. We present our experience with the diagnosis and management of cystic fibrosis in children seen at Post-Graduate Institute of Medical Education and Research, Chandigarh.