Infantile myofibromatosis is a rare disease characterized by myofibrob
lastic proliferation, and typically occurs in early infancy. There is
a wide spectrum of clinical presentation, which may involve various ki
nds of tissues in the body, Skin and subcutaneous lesions were the typ
es of tissue most often seen. Although a multicentric form may behave
aggressively, a solitary form of the tumor is benign with the possibil
ity of spontaneous regression. Conservative management is justified af
ter proper pathological diagnosis if the tumor involves an aesthetical
ly important area. A case bf solitary infantile myofibromatosis involv
ing the upper lip is presented. Partial excision for biopsy was perfor
med and long-term observation was undertaken. The tumor disappeared 3
years postoperatively.