Summary We report a case of a white girl with an ulcerated giant congenital melanocytic naevus that initially had a hard, stony consistency but in which the pigmentation and the induration are progressively vanishing. Very few cases of this variant of GCMN, known as sclerodermoid GCMN or desmoplastic hypopigmented hairless naevus, have been reported to date, and clinical evolution seems to be heterogeneous. We review the published cases and propose the term ‘sclerotic hypopigmented GCMN as a common descriptor of this type of congenital melanocytic naevus.