A male infant had three nodules on the left cheek, right forearm, and right neck. Biopsy specimen revealed infantile myofibromatosis (IM). Further evaluation revealed a solitary pulmonary nodule in the right middle lobe located far from mediastinal structures, which had no evidence of tumor enlargement on follow-up tomographic scan. Immunoperoxidase studies were negative for desmin and positive for actin. This is a potentially life-threatening multiorgan system disease; however, if lesions are limited mainly to the skin and soft tissues, the prognosis becomes more favorable. Although many reports suggest spontaneous and complete healing of the cutaneous lesions, our patient had profound atrophy at lesion sites as resolution occurred.