Benign tumorous condition can be encountered at very unusual location in oral cavity and pharyngeal region, which leads diagnostic difficulty. Here we describe a very unusual presentation of polypoid hamartoma on the root of the tongue. A 59-year-old woman presented with a polypoid tumor mass on the dorsal root of the tongue. Microscopically, it was hamartoma showing normal salivary glands of mucinous and serous types, lymphoid hyperplasia, and skeletal muscle bundles. Major differential diagnoses include accessory tongue, adenomatoid hyperplasia, and idiopathic hyperplasia of sublingual glands. To our knowledge, this is the first report of hamartoma arising in the root of the tongue presenting as polypoid mass. Correct diagnosis based on pathologic examination is essential for proper treatment.