Infantile myofibromatosis is a rare mesenchymal disorder that occurs predominantly in infancy and early childhood, in either solitary or multicentric form. It can affect soft tissue, muscle, skeleton, and occasionally, visceral organs. Infantile myofibromatosis without visceral involvement frequently undergoes spontaneous regression. Multicentric infantile myofibromatosis with involvement exclusively of the calvarium is extremely rare. We report an 8-month-old girl who presented with multifocal calvarial lesions. The child underwent total excision of the temporal mass, and histopathological study gave a diagnosis of infantile myofibromatosis. Serial follow-up by neuroimaging was obtained at 3, 6, 12, and 24 months postoperatively. Three months after surgery, a new lesion in the midline of frontal bone was found, and there was partial regression of the occipital lesion. Complete regression of the untreated lesions was shown at 24 months. Illustrated by our patient and literature review, we emphasize the importance of recognition and proper intervention for this rare, nonmalignant disorder.