A 4-year-old Japanese girl with a nonpigmented nodule on the anterior portion of the palate since birth is described. The overall microscopic appearance of the lesion was very similar to that of Spitz nevus of the skin. Diagnosis of Spitz nevus (mixed epithelioid cell and spindle cell nevus) was made on the basis of the clinical and histologic criteria for differentiating this lesion from malignant melanomas and common compound nevi. Positive immunostaining of epithelioid and spindle cells for S-100 protein and neuron-specific enolase was also indicative of their nevocellular nature. Review of the cases of Spitz nevus from the literature revealed that the present case most probably represents the first reported instance of this type of nevus in the oral cavity.