According to current knowledge the term giant congenital naevus is generally applied to skin alterations which consist of naevus cells, that are already conspicuous at birth and reach a diameter of at least 20 cm or more in adulthood. Surgical removal of such alterations is fundamentally indicated because there is high potential for degeneration. The surgical challenge is the functional and aesthetic reconstruction after removal. This article presents the incidence, natural course and pathology of such giant congenital naevus alterations. The advantages and disadvantages of various reconstruction procedures are presented together with an algorithm for management of these potentially malignant alterations.