Objective: Inverted papilloma (Schneiderian-type papilloma), involving the middle ear and mastoid as a primary lesion or as an extension of a sinonasal papilloma, is an extremely rare occurrence. This study was designed to report this rare case along with review of literature and to aware ENT surgeons regarding Inverted papilloma of middle ear and mastoid which is characterized by recurrence and local aggressiveness. Material and Methodology: This is a case of 23 yrs. old male presented with the complaints of left ear discharge with granulation in middle ear cleft. Patient underwent canal wall down mastoidectomy with complete removal of disease. Histopathological report revealed nonmalignant Inverted papilloma of middle ear. Observation and Results: Patient was followed for two years and no recurrence was found. Conclusion: Long-term follow-up after removal of inverted papilloma of the middle ear and mastoid is mandatory as the malignant transformation rate is high (35.3%) much more than that of paranasal sinuses.