Epidermal nevi are cutaneous hamartomas developing from ectoderm and one of their types is verrucous epidermal nevus. During the neonatal period, they are pink or slightly pigmented and velvety. Later, they darken and the surface become more warty. When lesions are distributed on one side of body, it is termed nevus unis lateralis. Lymphangioma is a lymphatic malformation which manifests with fluid- filled vesicles bulged on the skin surface.We present a 30- year – old man with pigmented, hyperkeratotic plaques on right side of his body associated with clear vesicles predominant at right side of his natal cleft. The histopathologic report of above lesions were epidermal nevus and lymphangioma, respectively.