Abstract: Leukoderma in association with congenital melanocytic nevi is a rare phenomenon; nevertheless several reports have been published in the literature. We present a 15-year-old boy born with a pigmented lesion on the lower and upper eyelid diagnosed as a congenital divided nevus of the eyelid. At the age of 13, he developed a depigmented area around the nevus and was diagnosed at first as having a halo nevus in a congenital nevus. Over the next two years, an area of depigmentation appeared around the contralateral eye. At the present time, the patient has bilateral periocular depigmentation. Congenital divided nevus of the eyelid is a rare lesion, and no reports have been published to date of depigmentation in association with this lesion. Our patient presented with depigmentation around the nevus as in a halo nevus phenomenon, although at the present time, the depigmented area has a symmetric periocular distribution, and therefore can be labeled as periocular vitiligo associated with a congenital divided nevus.