Harlequin baby may represent the extreme form of lamellar ichthyosis or may be a distinct entity. It is inherited as an autosomal recessive trait, but it's cause is unknown, although the abnormalities of keratinization and epidermal lipid metabolism have been suggested. We recently experienced a case of harlequin baby in a male neonate who showed the typical skin appearance at birth. His skin was markedly thickened and cracked, and large horny plates were formed over the entire body. His face was disfigured, and the nose and ear were flattened by the thickened skin. The chemosis and severe ectropion obscured the orbits, and his lips were everted and gaping. The inelastic skin resulted in flexion of all joints, and hands and feet appeared fixed and ischemic. The pathologic findings of skin biopsy were compatible to the lamellar ichthyosis and his family history was nonspecific. We managed the patient with supportive care for his special skin lesion, along with the trial of retinoic acid. However, the treatment was withheld and the baby was discharged against medical advice as the parents wised, and the baby died 5 days after discharge.