Background and aim Giant congenital melanocyticnevus (GCMN) is a pigmented lesion present at birth with wide extent on the skin surface. This study aimed to assess the management of these lesions, which remains controversial and needs to take into consideration the perceived risk of melanoma, the patient’s age, the cosmetic outcome, the surgical complexity, and the anesthesiological risk.

Methods This was a retrospective analysis and review of all records of children observed at the Paediatric Surgery Department oh the University-Hospital of Ferrara between 1991 and 2011 and treated for GCMN.

Results Twelve patients (median age 7 years, range 0.5–14) with GCMN were reported during the study period. Neurocutaneous melanosis was documented only once. Four patients underwent staged excision with grafting, two dermabrasion,and six implantation of skin dilator-expanded flap transfer. 2/12 lesions were macroscopically completely excised. Compliance to therapy was close to 100%. metastatic melanoma from an unknown primary site and death occurred in 1/12 (mean follow-up, 14 years).

Conclusions Decision making process to produce a final treatment choice for GCMN can be really complex. Regardless the outcome of therapy, a close regular post-operative follow-upto prevent or exclude possible complications proved to be useful in children with GCMN.