Neuroendocrine adenoma, also commonly known as carcinoid tumor, or adenomatoid tumor is a tumor which arises from middle ear and is a benign glandular neoplasm showing histologic and immunohistochemical neuroendocrine and mucin-secreting differentiation. Adenocarcinoid tumors show intracytoplasmic and luminal mucinous material highlighted by a Periodic acid-Schiff, while paraganglioma does not. Immunohistological markers plays an important role; in middle ear adenocarcinoid tumors, inner luminal cells positive for CK7 and outer basal cells positive for chromogranin, synaptophysin, and CD56. Paragangliomas are positive for chromogranin, synaptophysin, neuron specific enolase, serotonin, neurofilament and neural cell adhesion molecules with S-100 protein negativity. The authors present a 24-year-old male presented with left external auditory canal mass, initially diagnosed clinically with adenoma. Mass excision via external auditory canal approach with type 1 tympanoplasty was performed, and pathological and immunohistological studies revelaed paraganglioma. Literature concerning the subject is reviewed.