Introduction: Heterotopic Brain and Encephaloceles of middle ear usually present with symptoms of ear discharge and hearing loss. Some patients have additional symptoms of headache, abnormal ringing sensation and vertigo. Radiology may or may not show a communication with central nervous system in encephaloceles and the bony defect may be unnoticeable or attributed to the thinning of bone due to chronic otitis media. On computerised tomography, no distinction may be made between fluid, granulations, cholesteatoma and other space occupying lesions. Morphologically, it may be identified grossly if it presents with its characteristic pink colour and typical convoluted cerebriform pattern (CCP).  Methods: A 26 years female presented to us with unilateral ear discharge since birth and unilateral hearing loss on ipsilateral side for last 18 months. Clinically, radiologically and morphologically it mimicked chronic otitis media with cholesteatoma with no pre-operative and intra-operative distinction from the later. However, the coronal section at the level of anterior tegmen showed a small defect of the tegmen. T2 weighted MRI confirmed presence of brain tissue in middle ear and mastoid cavity. Results: Combined Middle Cranial Fossa and mastoid approach were employed, unviable brain tissue was excised and sent for histopathology. Viable brain tissue was reduced and tegmen defect was closed with temporalis muscle, conchal cartilage and temporalis fascia. Histopathology revealed glial tissue, ependyma and choroid plexus without any cholesteatoma. Conclusion: Tympanic encephalocelesare very rare and may closely mimic chronic otitis media. It may become very difficult to differentiate between the two which may lead to unexpected complications andloss