Communicating bronchopulmonary-foregut malformation, a variant of bronchopulmonary sequestration, is a rare anomaly characterized by communication between an isolated portion of the respiratory tree and the gastrointestinal tract. We report herein a unique case involving a 43-year-old man with squamous cell carcinoma arising in communicating bronchopulmonary-foregut malformation. This patient had a workup for a chief complaint of exacerbation of constitutional dysphagia, resulting in detection of squamous cell carcinoma involving the lower esophagus. Under the clinical diagnosis of esophageal carcinoma, esophagectomy was performed after neoadjuvant chemoradiotherapy. Pathologic findings showed that squamous cell carcinoma had arisen in malformed bronchopulmonary tissue constituting part of the distal esophagus segmentally. This case was unique in that squamous cell carcinoma developed in an extremely rare type of congenital abnormality that had functioned as a passageway for food from birth, as a result of chronic irritation for more than 4 decades.