Four cases of hypomelanosis of Ito with typical skin depigmentation and various noncutaneous findings were described. This neurocutaneous syndrome has been well documented by clinical investigations, especially computerized tomography and magnetic resonance imaging of the brain. The literature is surveyed giving special consideration to related neurologic manifestation. The most frequent neurologic abnormality was psychomotor retardation. Hearing loss in two patients and varicosis in one patient was striking. In one more patient, the symptoms resembled neurodegenerative disease.