Introduction: Adenocarcinoma of the esophagus and cardia is a rare cancer, associated with chronic reflux disease. Its associated mortality is still very high, reflecting both aggressive biology and lack of adequate treatments. The aim of this article was to describe up to date management of these complex tumors. Materials and Methods: A systematic review of the literature was performed, using PubMed Central database. Articles published after the year 2000 were included, with no language exclusion. Results: Reflux disease and Barrett esophagus are strongly associated with esophageal adenocarcinoma. A strict surveillance should be initiated at diagnosis. Both proton pump inhibitors and antireflux surgery failed to influence the incidence of cancer. Surgery and multimodal therapies are keystones for curative treatment, but no clear consensus exists for the best option. A clear trend in standardization of the surgical approach is observed since last ten years. However, the optimal approach for the tumors of the cardia is still not completely set. Complication rate is still high, but real progresses are made, through the implementation of less invasive techniques. Conclusion: Progress has been made in the management of esophageal cancer. However, the multiplicity of choices failed to lead to standardization. The development of international consensus regarding multimodal treatment and surgical approaches is needed.