Objectives: To describe the patterns of growth, nutritional status, body composition, and resting energy expenditure (REE) in prepubertal children with Alagille syndrome (AGS) before the onset of end-stage liver disease. Study design: Thirteen prepubertal subjects with AGS (8 male; mean age, 6.8 ± 2.8 years) were evaluated for growth parameters, body composition by skinfolds and by dual-energy x-ray absorptiometry, and REE by indirect calorimetry. The children with AGS were compared with a healthy, age-matched reference group of 37 prepubertal children. Results: Compared with healthy children, children with AGS had significantly reduced (P < .05) growth (weight, weight z score, height, height z score), nutritional status (midarm circumference, triceps skinfold, and midarm muscle area), and body composition (fat mass and fat-free mass). Subscapular thickness, percent body fat, and REE were not different. The AGS subgroup (n = 4) with REE greater than 110% predicted value had a reduced percent body fat (P < .02). Conclusions: Growth and body composition abnormalities are common in prepubertal children with AGS. (J Pediatr 1999;134:172-7)