Heterotopic brain tissue is an unusual substance which is very difficult to diagnose correctly preoperatively. We report on a 7-month-old male infant with heterotopic brain tissue of the soft palate, and describe the results of preoperative examinations including brain scintigraphy, angiography, brain computed tomography and magnetic resonance imaging (MRI). MRI was found useful to show that the tumor had no junction with the intracranial space. The patient was treated successfully without complication or recurrence after 1.5 years follow-up.