Giant congenital melanocytic nevus (GCMN) is defined as a melanotic lesion of the skin that is present at birth and is mainly characterized by a large area of the lesion, infiltration of melanocytes deep in the skin or subcutaneous tissue, with unique histological findings. Most of the patients are sporadic, but familial cases have also been reported, its pathogenesis is still unclear. It often places a serious psychological burden on patients and their families because it severely affects the appearance and may develop into malignant melanoma or neurocutaneous melanosis(NCM). The treatment methods of GCMN include surgical treatment and non-surgical treatment. Different methods have their indications and advantages and disadvantages. The prevention and treatment effect of GCMN are not satisfactory at present. Some studies have preliminarily shown that drug treatment is expected to reduce the incidence of complications and improve the recovery of patients, which can be used as an adjuvant therapy of traditional treatment methods.