Essential fatty acids (EFA) have proved to be important for normal bone mineral density (BMD) and bone growth in animal studies. Patients with cystic fibrosis often have low serum EFA levels, and low BMD has also been reported in patients with normal anthropometry. The aim of the present study was to analyse if BMD during a 2-year period was related to fatty acid status in patients with cystic fibrosis. Fifty-four patients, aged 6–33 years, were studied prospectively. BMD was measured with dual X-ray absorptiometry, and fatty acid concentrations in serum phospholipids were determined with capillary GLC. The cystic fibrosis patients showed low linoleic acid concentration and a high arachidonic acid (AA):DHA ratio in serum. The high eicosatrienoic acid:AA ratio, an indicator of EFA deficiency, increased further over 2 years, as did the total concentration of saturated fatty acids. In the adults there were no significant changes in fatty acids during the study. In the children, positive correlations were found between palmitic acid and bone mineral content in the lumbar spine and femoral neck. The lumbar spine BMD Z score correlated negatively with the AA:DHA ratio. No correlation was seen in adults except for a positive correlation between EFA deficiency index and the areas of lumbar spine and femoral neck. The present results imply that fatty-acid status influenced BMD in cystic fibrosis children, but not in adults, indicating that fatty-acid status wouldbe important for bone growth.