Infantile myofibromatosis is a rare benign tumor of childhood characterized by a myofibroblastic tumor in the skin, subcutaneous tissues, bones, and, occasionally, the viscera. The tumor may be solitary or multicentric. A solitary skeletal lesion most commonly occurs in the craniofacial region. We report a case of solitary infantile myofibromatosis of the skull, confirmed by excisional biopsy.