Aim: To investigate the carnitine status and the effect of carnitine supplementation on serum lipid profiles in children with hyperlipoproteinaemia, a clinical open trial was conducted at Hacettepe University Ihsan Dogramaci Children's Hospital, Section of Nutrition and Metabolism. Methods: Patients were given carnitine at a dose of 100 mg/kg/d for 12 wk. Blood samples for the determination of lipid profile and carnitine levels and urine samples for carnitine levels were obtained on admission, at week 4 and week 12 of the study period. Results: A total of 41 children were enrolled in the study: 20 patients had type II heterozygotes, eight patients had type II homozygotes, three patients had type III, six patients had type V and four patients had secondary hyperlipidaemias. Serum and urine carnitine levels were within normal limits on admission. No significant correlations were found between serum carnitine levels and serum lipid profiles. Serum HDL and apolipoprotein A-I decreased significantly during the 12 wk of intervention in type II heterozygotes. In type II homozygotes, total cholesterol and LDL levels at weeks 4 and 12 increased significantly compared to initial levels. No significant change was noted for lipid parameters in hyperlipoproteinaemia type V.