In this prospective study of 11 children with end-stage chronic liver disease (ESCLD), 36% were sarcopenic despite having normal fat mass indices, indicating a dissociation between lean and fat mass preservation. Body composition was associated with length of hospital stay after liver transplantation but not with graft function or survival at one year. The findings suggest that fat mass preservation may be a metabolic adaptation in pediatric CLD and that regional (appendicular) lean mass assessment is more accurate than whole-body measures due to organomegaly.