OBJECTIVE:To explore the level of coping and management of parents of children with inherited metabolic disorders (IMD) and the relationship with children's cognitive, behavioural and social functioning. METHODS:Parents of children (n = 22) with confirmed IMD (glutaric aciduria type I, methylmalonic aciduria, propionic aciduria, isovaleric aciduria, glycogen storage disease, maple syrup urine disease, ornithine transcarbamylase or very long-chain acyl-CoA dehydrogenase deficiency) completed standardised questionnaires regarding psychological distress, coping and family management. Children completed cognitive assessments and parents rated their behavioural and social functioning on standardised questionnaires. Scores were compared with normative data. RESULTS:Most parents were coping well; 4/22 reported high levels of psychological distress. Exploratory analysis found that parent coping variables were correlated to the child's internalising symptoms, whereas family management was related to children's externalising behaviours and social skills. No relationship was found between parent variables and cognitive functioning. CONCLUSIONS:Parental coping and family management impact on the child's internalising symptoms and externalising behaviours, respectively. Early identification of issues in these domains may enhance referral for therapeutic interventions and family support programmes.