Basaloid-squamous carcinoma is a poorly-differentiated variety of squamous cell carcinoma. The most common location is the upper aerodigestive tract and the incidence of this entity in the esophagus is low. Despite advances in knowledge of the biology of these tumors, their recent description and their low frequency has hampered consensus on the therapeutic approach. We present a case of basaloid-squamous cell of the esophagus a 54 year-old man who underwent an esophagectomy with lymphadenectomy and has been followed-up for 5 years with no evidence of disease recurrence. We also provide a literature review of this entity.