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Fibromatoses are benign fibroblastic proliferation, which forms infiltrating tumor-like mass. Their histologic features cannot reflect the biologic behavior; local recurrence and aggressive infiltration to the adjacent tissue make fibromatoses intermediate tumor, between benign and malignant. Infantile fibromatosis consists of desmoid-type tumor and lipofibromatosis, and mainly affects children under age of 10. While desmoid-type tumor is characterized by elongated mature fibroblastic proliferation, the fibroblasts in lipofibromatosis look more primitive and accompany adipocytes. Here, we report a case of infantile fibromatosis of 4 month old boy involving the tongue.