Infantile myofibromatosis is an unusual condition generally presenting in the newborn period. The case being reported is that of a female newborn who had multiple lesions that involved skin, subcutaneous tissue, skeletal muscles, bone, and lungs. The disease was diagnosed because of the easily palpable skin tumors and subcutaneous nodules that were obvious immediately after birth. The diagnosis was established by histopathological examination of one nodule that showed a spindle-celled mesenchymatogenic lesion demonstrating the morphological and immuno-phenotype characteristics of myofibroblastic differentiation. The histologic picture, combined with the clinical manifestations and the imaging findings, are consistent with infantile myofibromatosis. The physical condition of the newborn was excellent and remains so six months later. The tumors of the skin and the subcutaneous nodules have gradually regressed without therapy. At the age of six months, four (4) nodules are palpable; the infant is under continuous observation.