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Although halo nevus (HN) is frequently observed, the relationship between vitiligo and HN in children has rarely been investigated.To investigate the association between HN and vitiligo in children and understand if HN/HNi might be a risk factor for vitiligo.Ninety-eight patients with only HN/HNi and 27 with HN/HNi and vitiligo were investigated for number and localization of HN/HNi, family history for HN/HNi and vitiligo and personal and family history for autoimmune or other diseases. A follow-up telephone interview was performed to investigate the evolution of HN/HNi and the possible onset of vitiligo and/or other diseases.In the HN/HNi and vitiligo group, HN/HNi and vitiligo had started almost simultaneously in 11 children; in nine, the onset of HN/HNi was followed by vitiligo after 6 months to 5 years; seven patients presented vitiligo first and HN/HNi after 3–9 years. Patients with associated vitiligo had, significantly more often, multiple HNi and a positive personal and/or family history of autoimmune thyroiditis compared with those with only HN/HNi. Follow-up longer than 5 years was available in 54/98 patients with only HN/HNi; two of them, both with multiple HNi, developed vitiligo. After follow-up, multiple HNi were noticed in 18/52 patients without vitiligo and in 9/11 of those in whom HN/HNi heralded vitiligo (s.s.).In patients with multiple HNi, the risk of vitiligo and other autoimmune diseases seems to be higher than in pediatric patients with a single HN; clinicians should pay particular attention to children with multiple HNi and personal or family history of autoimmune diseases.