Halo congenital nevus is a condition in which whitish halo is formed in association with congenital melanocytic nevus (CMN). It is rare phenomenon in CMN and halo nevus and vitiligo were postulated to have a common immunologic etiology. A 3-year-old girl presented with a hyperpigmented patch on right lower leg since birth. The nevus was measured as 9 x 6 cm, and had hypertrichosis on surface. Dermoscopic findings showed whitish globules with poliosis. After 3 years, a hypopigmented halo and partial regression of the lesion were observed. A month later, additional hypopigmented patches were noted on abdomen that were consistent with vitiligo. When the child became 9-year-old, complete regression of the nevus with depigmentation was observed and additional hypopigmented patches were noted on right hand. To our knowledge, halo congenital nevus with extra-lesional vitiligo was uncommon condition and around 20 cases were reported in the literature. Moreover, this is unique in the respect of the complete regression of CMN that started inside of nevus as halo phenomenon related to vitiligo. Encountering congenital melanocytic nevus with halo formation, the possibility of complete regression might be considered like the present case.