Leiomyosarcoma of the esophagus is an uncommon neoplasm of mesenchymal origin. The simultaneous occurrence of leiomyosarcoma and squamous cell carcinoma in the esophagus as separate tumors is a very rare event. We present a case leiomyosarcoma associated with squamous cell carcinoma that was diagnosed after surgical resection. The clinical, radiologic, endoscopic and pathologic findings of this rare entity, and four additional cases previously reported in the literature are reviewed.