A 21-year-old male visited our hospital with a complaint of aggravating dysphagia and odynophagia for a few days.Esophagogastroduodenoscopy showed huge bulging mucosa with an intact surface causing luminal narrowing at 35 cm from the incisor teeth.Endoscopic ultrasonography showed an about 35 mm sized irregular margined in-homogenous hypoechoic lesion with an obscure layer of origin.Endoscopic ultrasonography fine needle aspiration revealed spindle cell proliferation without immunoreactivity for CD117,SMA,and cytokeratin.The patient underwent excision of the subepithelial lesion at the distal esophagus.On pathologic examination of the specimen,the tumor was composed of short fascicles of oval to spindle cells with eosinophilic and clear cytoplasm and vesicular nuclei.The tumor cells were positive for S-100 and SOX10and negative for CD117,SMA,HMB-45,melan-A,cytokeratin,and CD99.The split-apart signal was detected in EWSR1 on FISH,suggesting a malignant gastrointestinal neuroectodermal tumor.At the time of writing,the patient is on radiation therapy at the operated site of esophagus and doing well,with no recurrence for three months.Malignant gastrointestinal neuroectodermal tumor is a rare gastrointestinal tumor with features of clear cell sarcoma,without melanocytic differentiation,and shows a poor prognosis.This is the first reported case of malignant gastrointestinal neuroectodermal tumor arising as subepithelial lesion in the esophagus. A 21-year-old male visited our hospital with a complaint of aggravating dysphagia and odynophagia for a few days. Esophagogastroduodenoscopy showed huge bulging mucosa with an intact surface causing luminal narrowing at 35 cm from the incisor teeth. Endoscopic ultrasonography showed an about 35 mm sized irregular margined in-homogenous hypoechoic lesion with an obscure layer of origin. Endoscopic ultrasonography fine needle aspiration saw spindle cell proliferation without immunoreactivity for CD117, SMA, and cytokeratin. the patient underwent excision of the subepithelial lesion at the distal esophagus. On pathologic examination of the specimen, the tumor was composed of short fascicles of oval to spindle cells with eosinophilic and clear cytoplasm and vesicular nuclei. tumor cells were positive for S-100 and SOX10 and negative for CD117, SMA, HMB-45, melan-A, cytokeratin, and CD99. The split-apart signal was detected in EWSR1 on FISH, suggesting a malignant gastrointestinal neuroectodermal t umor. At the time of writing, the patient is on radiation therapy at the operated site of esophagus and doing well, with no recurrence for three months. Malignant gastrointestinal neuroectodermal tumor is a rare gastrointestinal tumor with features of clear cell sarcoma, without melanocytic differentiation , and shows a poor prognosis.This is the first reported case of malignant gastrointestinal neuroectodermal tumor arising as subepithelial lesion in the esophagus.