We report a case of a congenital, melanocytic nevus showing spontaneous regression. At birth, a pigmented lesion, 4 × 6.5cm, was already present on the scalp. At the age of 20 days, the center of the lesion became crusted with a purulent discharge. Following healing of the inflammed lesion, the nevus showed a tendency to regress, starting from the previously inflammed site. At the age of 11 months, the nevus showed marked regression, leaving an atrophic and depigmented center with pea-sized papules and a thin, pigmented rim. Skin sections taken from the central depigmented area showed marked fibrosis and scanty nevus cell nests in the dermis. Histological examination of the pea-sized papule in the center of the lesion repealed large epithelioid cells with abundant cytoplasm dispersed between the collagen fibers; it resembled a spindle cell and epithelioid cell nevus with epithelioid cells predominating. There were no clinical or histologically malignant changes.