Prominent lymphocytic infiltration and lymphoid follicles surrounding tumor cells are extremely rare findings in esophageal carcinoma. We report on the endoscopic, histological, and immunohistochemical features of a rare case of squamous cell carcinoma of the esophagus with lymphoid stroma. A 59-year-old woman was diagnosed with type 0-Is superficial esophageal carcinoma and underwent radical esophagectomy with lymph node dissection. Macroscopically, the tumor was protruding, and was covered with normal epithelium resembling a submucosal tumor. Histopathological examination demonstrated that the esophageal lesion was classified as a poorly differentiated squamous cell carcinoma with lymphoid stroma, extending to the deep submucosa (SM3) with lymph node metastasis (T1b, N2, M0, stage II). Epstein-Barr virus infection was ruled out by immunohistochemical and in situ hybridization analyses. Infiltrating B-lymphocytes were observed forming lymphoid follicles adjacent to carcinoma cell nests, and numerous T lymphocytes were widely spread throughout the specimen, as well as distributed in the marginal zone of the lymphoid follicles. Prominent human leukocyte antigen DR region (HLA-DR) immunoreactivity was noted in most carcinoma cells and focally infiltrating B cells in the lymphoid follicles, and these observations were thought to be due to activation of immunological interactions between carcinoma cells positive for HLA-DR and host lymphocytes.