Objective To investigate the clinical manifestations, diagnosis and treatment of middle ear adenomatous neuroendocrine tumors (MEANTs) and improve the understanding of MEANTs. Methods The clinical data of 2 cases of MEANTs admitted to the Department of Otolaryngology, Zhongshan Hospital Affiliated to Xiamen University from 2020 to 2022 were retrospectively analyzed, including preoperative endoscopy, hearing examination and imaging examination, postoperative pathological diagnosis data and postoperative follow-up. Results All two cases were male.One MEANTs occurred on the right side，another occurred on the left side.Endoscope examination of one case revealed that the right external ear canal was filled with reddish polypoid tissues, tympanic membrane was not possible to visualize.Another case presented with complete bulging of the tympanic membrane.In temporal bone CT, the soft-tissue mass located the middle ear was found to extend to the antrum and the external auditory canal.MEANTs tend to surround the ossicles without erosion.MRI images showed equalT1and T2 signals with intensifying in tympanum.Two patients underwent canal wall down mastoidectomy and ossicular reconstruction.Both cases showed adenoid or solid nest-like structures in the pathological morphology. Immunohistochemistry showed different levels of CK-P and neuroendocrine markers. Two patients showed no evidence of recurrence during followed up.Conclusions MEANTs are rare middle ear masses with benign morphological features and its clinical manifestations are not typical.MEANTs surgical resection of the tumor is the main method of treatment.Regular follow-up of the patients is required after surgery.