An extrarenal malignant rhabdoid tumor of the tongue in a 10-day-old boy is described. The tumor produced severe respiratory distress and had an aggressive clinical course. The patient died 17 days after the initial diagnosis. The tumor was mostly composed of poorly differentiated, round, polygonal or elongated cells. Ultrastructural examination showed intracytoplasmic filamentous inclusions in many of the cells. The cytoplasm was diffusely positive to anti-vimentin antibodies with focal, scarce positivity to anti-keratin antibodies. To the best of our knowledge, this is the first reported case of malignant rhabdoid tumor of the oral cavity.