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Infantile myofibromatosis is a rare disorder of mesenchymal cell proliferation that can affect the skin, bone, muscle, and viscera. We present a case of a 6-week-old male with a rapidly enlarging congenital solitary infantile myofibroma. The differential for congenital tumors of the head and neck is broad, and thorough evaluation is required to rule out life-threatening malignancy. Currently, there is no first-line imaging modality of choice to assess for skeletal and/or visceral involvement in patients with infantile myofibromatosis. We recommend the use of whole-body magnetic resonance imaging (MRI), as it quickly provides detailed information regarding extent of disease and does not expose the patient to the harmful effects of radiation.

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