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Pathological gambling (PG) is distinguished in both Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) and the tenth edition of the International Classification of Mental and Behavioral Disorders (ICD-10) from gambling secondary to mania and from social gambling, which does not persist when adverse events occur. It may be associated with dopamine agonist treatments for Parkinson's disease. PG is also associated with greater health problems and increased use of medical services. Although cognitive-behavioral therapy (CBT), opioid antagonists, and sustained-release lithium carbonate appear promising for the treatment of PG, several limitations affect the current body of knowledge. PG is a common disabling psychiatric disorder associated with high rate of co-occurring disorders, particularly substance use disorders, and high rate of social and occupational dysfunction. Although psychotherapies and pharmacotherapies have shown promise, the limited data preclude making treatment recommendations with substantial degree of confidence.