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This systematic review of 31 studies examines the quality of life of adult congenital heart disease patients and the relationship between disease severity and quality of life. The results suggest that these patients' quality of life is compromised in the physical domain compared to their healthy counterparts, but no differences were found in the psychosocial and environmental/occupational domains. Methodological limitations were identified, and the review highlights the need for future studies to address these limitations to provide robust evidence and valid conclusions. Approximately 8 in 1000 children are born with congenital heart disease, and due to advances in treatment, 90% are now expected to survive into adulthood. The review aimed to describe the quality of life of adult patients with congenital heart disease, examine the relationship between disease severity variables and quality of life, identify the quality of the existing literature, and suggest areas for future investigations.