[Congenital giant blue nevus. Apropos of a case]

A case of a three years old girl showing a congenital giant blue nevus, in the neck and the occipital region, is presented. The giant blue nevus was associated to an important cutaneous nevomatosis. The treatment has consisted of an surgical excision of an surgical excision of the main lesion, and operative continuations have been simple. The literature study showed 11 cases of congenital giant blue nevus and demonstrate the high risk of malignancy, and the importance of an early surgical treatment.