Hypomelanosis of ITO (HI) is a heterogeneous and complex nearocutaneous disorder affecting the skin, brain, eye, skeleton, and other organs. The skin findings are distinctive and, in fact, are.the only constant feature of HI; hypopigmented whorls, streaks, and patches tend to follow Blaschko's lines. Blaschko's lines form a V-shaped pattern over the back, an S-shaped pattern over the anterior trunk, and linear streaks over the extremities.HI was first described by ITO as incontinentia pigmenti achromians, but it is now commonly known as HI to avoid confusion with incontinentia pigmenti. HI affects male and female subjects equally and is usually a sporadic disorder with minimal recurrence risk.Our patient was a 17-year-old boy who came to our clinic with tonic – clonic seizures since he was 5. He had aura before seizures as epigastric pain and facial numbness. Past medical history and family history of patient was negative. There was short of stature and macrocephaly in his physical exam. There were also V-shaped hypopigmented lesions over the back and linear over the extremities.A systolic murmur of grade I-II was heard in aortic area and chest wall of patient had a pattern of pectus excavatum. Other physical exams were normal. In neuroimaging studies, in brain MRI, there were multiple periventricular white matter lesions that were very similar to MRI findings of multiple sclerosis. As a result of our findings, clinicians should consider HI as a differential diagnosis of diseases with periventricular white matter lesions especially multiple sclerosis.