Although liposarcoma is the second most common soft-tissue sarcoma in adults, its incidence within the gastrointestinal tract is distinctly low. Esophageal involvement is exceedingly rare and only four cases have been described so far. A fifth case is presented here along with a thorough review of the literature of polypoid lipomatous tumors of the esophagus. Diagnostic and therapeutical strategies of these tumors are discussed in detail.