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To assess the quality of life and psychosocial functioning among pediatric patients with Glycogen Storage Disease (GSD) types Ia and Ib.Thirty-one youth with GSD types Ia and Ib and 42 healthy controls participated. Quality of life ratings from the GSD types Ia and Ib sample were compared with a previously reported clinical comparison sample. Children completed measures of quality of life, loneliness, family functioning, and sibling relationship quality (e.g., warmth, conflict). Parents completed measures of parental distress, parenting stress, child adaptive behavior, and child emotional and behavioral functioning.Quality of life was generally lower in youth with GSD relative to healthy controls but similar to those with a chronic illness. Children with GSD were rated as having more internalizing symptoms, social problems, and lower independent functioning relative to healthy controls. Parents reported greater distress and parenting stress relative to healthy controls.The presence of GSD types Ia and Ib are associated with reduced quality of life and independent functioning, and elevated levels of internalizing distress and parental stress relative to healthy peers. Relative to their children, parents generally reported that their child was more impaired, which suggests the need for multiple informants during assessment and active parental involvement during psychological treatment. These points should be kept in mind when assessing and treating youth with this disease and their families as psychological interventions that target areas of concern (e.g., adherence, coping with having a chronic disease) may be helpful for improving child and family outcomes.