CASE REPORT: A 1-year-old girl presented with a 10-month history of progressive protuberance of the left frontal skull. Magnetic resonance imaging and computed tomography demonstrated a large osteolytic interosseous mass extending to the frontal sinus and temporal base without any intracranial invasion. A fronto-temporo-parietal craniectomy of the outer skull table and excision of an interosseous tumor resulted in local dural exposure in the temporal area that was covered by cranioplasty. Pathological examination identified desmoplastic fibroma (DF) of the skull. The patient's cranial asymmetry was improved without recurrence of the tumor up to the 12th month after excision. DISCUSSION: In the literature, 11 cases of DF of the skull have been reported, two of which have involved children (one an infant). We report the second known infantile case of DF of the skull.