Adenosquamous carcinoma (ASC) of the esophagus is an uncommon type of esophageal cancer that contains both adenocarcinoma and squamous cell carcinoma elements. Data on this biologically unique type of cancer are limited and mainly stem from case reports and small case series. We performed an audit of the available literature and synthesized a review on the epidemiology, pathogenesis, histopathology, clinical manifestations, diagnosis, treatment and prognosis of ASCs. Adenosquamous carcinoma of the esophagus is a rare type of esophageal cancer. Histological examination is necessary to confirm the diagnosis of ASC and patients usually receive multimodal treatment. Patient outcomes are not well defined and further research could help us better understand the pathophysiology and unique needs of patients with this rare malignancy.