Cornu cutaneum, or giant cutaneous horn (GCH), is an uncommon but disfiguring lesion that may cause the patient considerable distress. The aesthetic implications of such lesions are obvious. The etiology and treatment of GCH are more obscure, both to patients and physicians alike. We present a female patient of African descent with a GCH of the scalp, which represents the first such case to our knowledge outside Africa. The etiology and management of GCH in all races are reviewed.