Abstract Introduction Children with chronic liver diseases are exposed to biological and/or environmental risk factors that can compromise their neuromotor acquisition and development of functional skills. Objective To describe the neuropsychomotor development (NPMD) and functional skills of children with chronic liver diseases. Methods Cross-sectional, descriptive and exploratory study carried out with children up to 6 years old who were selected at a reference hospital in the state of Bahia, Brazil, from November 2019 to March 2020. Children in outpatient care with clinical, laboratory and histological diagnosis compatible with chronic liver disease were considered eligible. The instrument for assessing neuropsychomotor development was Denver II. Functional skills were obtained by applying the Pediatric Evaluation of Disability Inventory computer-adaptive test (PEDI-CAT) to parents or primary caregivers, Speedy version (Speedy-CAT). Results Of the 34 children with chronic liver disease, 52.9% were female, aged between 4 and 6 years (64%). The results of the Denver II test showed that 68.7% (22/32) of the sample were at risk for NPMD. In the PEDI-CAT, the scores of children with liver disease at risk for NPMD were 60.7 ± 9.1 in the daily activity domains, 57.6 ± 11.8 in mobility and 48.3 ± 6.2 in the social/cognitive domains. Conclusion Children with chronic liver disease are at risk for NPMD, although not presenting impaired functional skills when evaluated by the PEDI-CAT.