Partial unilateral lentiginosis (PUL) is a rare pigmentary disorder characterized by multiple lentigines on otherwise normal skin affecting one side of the body. We report on a girl with an extensive form of bilateral lentiginosis with systematized segmental distribution and a unilateral congenital cataract. The arrangement of the lentigines is reminiscent of a checkerboard pattern. The benign condition is discussed in the context of systemic diseases with lentigines.