Background: In order to effectively improve motor function, a sound understanding of one's bodyfor example, relative spatial position, relationships, names and functions of body partsis essential. The aim of this study was to explore how children with congenital lower limb deficiency (LLD) perceive their bodies, particularly their legs. Methods: Six children with congenital LLD and 14 controls, aged 5-12 years, were recruited for this study. They drew self-portraits and answered questions about names of body parts. These body part-related questions consisted of a production test, in which an examiner pointed to body parts on each child and asked the child to name them, and a comprehension test, in which the examiner mentioned body parts and asked the child to point to them on their own body. Results: No differences were found between the self-portraits of children with LLD and those of the control children. In the verbal tests, children with LLD responded correctly at lower rates to questions on body trunk, upper limbs, arms, hands and feet than the control children. Conclusion: Children with LLD have diminished lexical-semantic body knowledge of the upper limbs and feet compared with children without LLD.