ABSTRACT: Children with cystic fibrosis (CF) and pancreatic insufficiency (PI) are at risk for fatty acid (FA) abnormalities and essential FA deficiency, with low linoleic acid (LA) and docosahexaenoic acid (DHA) concentrations and abnormal triene:tetraene (T:T) and arachidonic acid (AA):DHA ratios. The aim of the article was to determine whether type of dietary fat predicted serum LA, DHA, T:T, and AA:DHA ratios in subjects with CF and PI as compared to an unaffected comparison group.Serum FA concentrations were assessed by capillary gas-liquid chromatography (mol%) and dietary intake by 7-day weighed food records; the 3-day coefficient of fat absorption was calculated. Total energy intake was expressed in kilocalories.A total of 65 subjects with CF and PI (8.4?±?1.0 years, 32 girls) and 22 controls (8.5?±?1.1 years, 13 girls) were included. Despite greater energy, saturated fat, and LA intake, the subjects with CF had lower serum LA and DHA and higher T:T and AA:DHA than those in the comparison group. Dietary total fat, monounsaturated fatty acid (MUFA), polyunsaturated fatty acid (PUFA), LA, total ? 6 polyunsaturated fatty acid (T?6PUFA), and ?-linolenic acid (ALA) intake positively predicted serum LA concentration. MUFA, total ? 3 polyunsaturated fatty acid (T?3PUFA), and ALA intake positively predicted serum DHA concentration. Total dietary fat, MUFA, PUFA, T?3PUFA, LA, and ALA intake negatively predicted serum T:T. ALA and T?3PUFA intake negatively predicted serum AA:DHA.Dietary fat patterns influenced serum LA, DHA, T:T, and AA:DHA in children with CF and PI. These data suggest that changes in dietary practices may result in FA profiles associated with improved clinical outcomes.