Delirium not induced by alcohol or other psychoactive substance and alcohol withdrawal delirium (or delirium tremens) are both cerebral syndromes with similar presentations and are associated with various adverse outcomes. Recently, interest in identifying genetic predisposing factors that influence the occurrence or the outcome of delirium has become a prominent point of delirium research. We systematically searched published articles concerning genetic associations and the occurrence and outcome of delirium. Of 33 identified articles, six investigated non-alcohol withdrawal delirium, and from those six, five evaluated an association with apolipoprotein E (APOE). One association of APOE genotype with the emergence of delirium and two associations of APOE genotype with the duration of delirium were reported. The remaining 27 identified articles investigated genetic associations with alcohol withdrawal delirium and were mainly related to dopamine. Two studies reported a significant association of alcohol withdrawal delirium with the dopamine transporter gene (SLC6A3) and the dopamine receptor 3 (DRD3). Results are inconclusive, and no hard evidence exists due primarily to insufficiently powered studies and other methodological issues. Prospective studies incorporating systematic and rigorous diagnostic criteria and involving long term follow up are needed to advance understanding of this field.