Schneiderian-type papilloma of the middle ear is a rare finding. We present a 46-year-old Aboriginal man with a large tympanic membrane perforation and a Schneiderian-type papilloma filling the middle ear. The aim of this study is to familiarize clinicians with this uncommon disease through discussion of its clinical presentation, diagnostic considerations and management. A search of English-language peer-reviewed literature was undertaken using the key words “Schneiderian-type papilloma,” “inverted papilloma,” and “middle ear.” A total of 29 cases (including the present case) of Schneiderian-type papilloma involving the middle ear were reviewed. Common presenting symptoms include hearing loss, otalgia, and otorrhea. Middle ear disease is associated with higher rates of recurrence and malignant transformation than its sinonasal counterpart. Radical surgical resection is the only curative treatment. Schneiderian-type papilloma is a benign, but locally aggressive, epithelial neoplasm most commonly arising in the sinonasal tract. Whilst involvement of the middle ear is extremely rare, knowledge of this condition is important due to its propensity to recur and the high rate of malignant transformation.