A 2-YEAR-OLD white boy had an 8-mm nodular lesion on his tongue since birth. The nodule was completely resected. No signs of recurrence were noted at 7-year follow-up. The specimen consisted of 2 mucous-covered fragments measuring 1 and 2 cm in diameter. Microscopically, there was a moderately well-circumscribed, 7 × 6 × 4 mm nonencapsulated, submucous nodular lesion (Figure 1). The central portion of the nodule was composed of thin, elongated cells with eosinophilic cytoplasm arranged haphazardly in slender fascicles or isolated. Small oval- to spindle-shaped undifferentiated cells with indistinct cytoplasm were interspersed among them (Figure 2). A decrease in the number of the undifferentiated cells was noted toward the periphery; the elongated cells became larger and disclosed a greater degree of cytoplasmic differentiation (Figure 3). Both types of cells showed bland nuclei with no mitoses and inconspicuous nucleoli. A discrete amount of myxomatous stroma were noted, especially in the central portion of the lesion. There were no areas of necrosis or inflammation. Immunohistochemical stains were obtained (Figure 4 and Figure 5).