A clinical and pathological review of 67 cases of congenital giant nevi and of 5 cases of malignant transformation in congenital giant nevi brings out the following. 1. The lesions should be regarded as premalignant. 2. An aggressive approach is advocated to remove the lesion before school age. 3. Staged excisions, combined with skin grafting and/or local rotation flaps, are advocated. 4. Any nodularity or ulceration developing within a congenital giant nevus should be regarded as an ominous sign. 5. A long-term follow-up is necessary in those patients having subtotal excisions.