Aim: The prognosis of anal adenocarcinoma is poor and the management remains controversial. We carried out a literature review to identify current practice in the management of anal adenocarcinoma. Method: A systematic review of the literature was performed for studies in the English language published between 1950 and 2011. All those that focused on the management of anal adenocarcinoma were considered for inclusion. Results: Sixteen retrospective observational studies were identified; no randomized trials were found. Most of the studies contained small numbers of patients due to the rarity of anal adenocarcinoma. Treatment included local excision), radiotherapy, chemotherapy, chemoradiotherapy and abdominoperineal excision. Most studies concluded that a multimodality approach, combining radical surgical resection with neoadjuvant/adjuvant chemoradiotherapy was the optimal management strategy. Conclusion: The prognosis of anal adenocarcinoma is poor, and there is little information on the optimal management. The relevant studies indicate that a combination of radical surgical resection and pre- or postoperative chemoradiotherapy offers the best chance of survival. © 2013 The Association of Coloproctology of Great Britain and Ireland.