Pulmonary tumor thrombotic microangiopathy (PTTM) is an uncommon cancer-related complication that has been most frequently reported to be associated with adenocarcinoma. We present a case of PTTM which developed in a 60-year-old man with esophageal carcinoma. One year after definitive treatment of the tumor, he developed pulmonary hypertension. Transbronchial lung biopsy (TBLB) specimens showed fibrocellular intimal proliferation and luminal stenosis of the small pulmonary vessels, which contained squamous cell carcinoma cells. Thus, PTTM associated with esophageal carcinoma was diagnosed. This is the first reported case of PTTM associated with esophageal squamous cell carcinoma. TBLB seemed to be useful for obtaining a definitive diagnosis.