The occurrence of these tumors in neonates and young children in the oral cavity and in particular in the tongue is rarely reported. Development of the benign tumor disease depends on the form of the tumor, which can be distinguished between a solitaire, a multiloculaire and a generalized form. In contrast to the other two forms, cases of generalized infantile myofibromatosis involve the visceral organs and have a poor prognosis with a mortality rate of 75 %. Spontaneous remission is often reported in the solitaire and multiloculaire varieties of infantile myofibromatosis.