Infantile myofibromatosis (IM) is a disorder characterized by proliferation of benign myofibroblastic tumors that typically manifest as solitary or multiple nodules in the skin, muscle, bone, subcutaneous tissues, and visceral organs and can pose significant morbidity and mortality risks, particularly in cases involving visceral organs or causing functional impairment. These soft tissue lesions present before age two and can undergo spontaneous regression or are amenable to surgical resection. Successful clinical management with low dose metronomic chemotherapy (Methotrexate and Vinblastine) is possible and can treat extensive disease as seen in our patient.