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To investigate body fat distribution and glucose and lipid metabolism in HIV-infected children with the aim of describing the lipodystrophic syndrome in children.Cross-sectional study including 39 HIV-infected children aged 3–18 years.Clinical lipodystrophy was defined as peripheral fat wasting (facial and/or buttock and/or limb atrophy with arm skinfold thickness lower than the third percentile of the reference values for sex and age) and/or truncal adiposity (breast enlargement and/or buffalo neck and/or relative abdominal obesity with trunk : arm skinfold ratio > 2 standard deviations). Fasting serum lipid concentrations were measured and an oral glucose tolerance test was performed.Of 39 HIV-infected children, lipodystrophy was observed in 13 children (33.3%): eight with truncal lipohypertrophy, three with peripheral lipoatrophy and two with combined lipodystrophy. Combined lipodystrophies were observed only in adolescents with a more severe presentation than in prepubertal children. Lipodystrophic children had higher fasting insulinaemia (7.0 ± 8.5 versus 3.0 ± 2.3 μU/ml;P = 0.07), suggesting a certain degree of insulin-resistance. Hypercholesterolaemia (23% versus 15%;P = 0.59 ) and hypertriglyceridaemia (15% versus 11%;P = 0.76) were observed with the same proportion in the lipodystrophic as in the non-lipodystrophic groups; 23% of the non-lipodystrophic children had dyslipidaemia.The lipodystrophic syndrome prevails in HIV-infected children in the three clinical forms initially described in adults but appears less severe before the initiation of puberty. Insulin-resistance occurs in lipodystrophic children only, whereas dyslipidaemia exists in non-lipodystrophic children as well, suggesting that dyslipidaemia could reflect subclinical alteration of the adipose tissue.