Incontinentia pigmenti is characterized by irregular linear blisters with erythematous lesions which are developed from birth or later, verrucous papules on the extremities which are noted after a few weeks or months, and the appearance of streaks, spots and swirls of hyperpigmentation. This disorder is known as a systemic disorder caused by a defect at the developmental stage of organs originated from ectoderm or mesoderm. We experienced an incontinentia pigmenti in a male newborn infant who did not have any family history and had cutaneous lesions, which were in bullous and verucous stages, ophthalmic problems, and neurologic abnormalities.