Atrial fibrillation (AF) is a life-complicating illness adversely affecting morbidity, health-related quality of life (HRQOL), and healthcare use. Studies using HRQOL instruments suggest that patients diagnosed with AF experience more psychological distress than do healthy controls. Psychological distress in forms of anxiety and depression in patients with heart failure or coronary artery disease is related to increased mortality, morbidity, and consumption of healthcare. However, there is a critical lack of knowledge regarding the type and extent of psychological distress and its consequences in patients diagnosed with AF. This article will review the current state of scientific knowledge regarding psychological distress in patients with AF and offer suggestions for future studies. Medline, CINAHL, PscyhInfo, and Psychology and Behavioral Sciences Collection databases up to June 2009 were reviewed for key terms atrial fibrillation, psychological distress, affective distress, mood, emotional distress, psychological stress, negative affect, anxiety, depression, anger, and hostility. Ten studies using tested instruments to measure psychological distress were retained. The prevalence of psychological distress was not consistently reported. Combined findings revealed that psychological distress in the form of depression and/or anxiety uniquely contributed to greater AF symptom severity, diminished HRQOL, and recurrence of AF. Studies describing interventions to address psychological distress were not found. Lack of conceptual clarity and diversity of study purposes, designs, participants, and instruments limit the ability to draw coherent conclusions from findings. Nevertheless, findings suggest that psychological distress is present in a substantial portion of patients diagnosed with AF and its presence is related to adverse outcomes. Further study to identify the prevalence, characteristics, and consequences of psychological distress in patients diagnosed with AF is required to extend our knowledge and provide a foundation for development of interventions to address psychological distress in this rapidly increasing population.