ABSTRACT.Carnitine metabolites (total, free, short and long chain) were analyzed in cord blood of cystic fibrosis (n=5), non‐CF siblings (n=7), and controls (n=8). Total acylcarnitine (short and long chain combined) was significantly lower (<0.001) in CF compared to both control groups. Total and free carnitine showed no significant differences between the three groups. These findings are compatible with disturbed fatty acid metabolism in utero and may be related to the increased energy expenditure characteristic of CF infant