Ito hipomelanozu, tek veya çift taraflı maküler hipopigmente sarmal şekiller, çizgiler ve yamalarla karakterize bir antitedir. Beraberinde gözlerin, kas iskelet sisteminin ve santral sinir sisteminin anormallikleri bulunabilir. Hemimegalensefali, bir serebral hemisferin tek taraflı büyümesiyle karakterize konjenital beyin malformasyonudur. Hemimegalensefali, genelde izole bir bulgu olmasına rağmen, birçok sendromla da birlikte tarif edilebilir. Ito Hipomelanozu ve hemimegalensefali birlikteliğini gösteren bir olguyu sunmaktayız. Anahtar Kelimeler: Ito Hipomelanozu, hemimegalensefali, yenidoğanHypomelanosis of ıto and hemimegalencephaly: a case report Hypomelanosis of Ito is characterized by unilateral or bilateral macular hypopigmented whorls, streaks, and patches. Abnormalities of the eyes, musculoskeletal system, and central nervous system may also be present. Hemimegalencephaly is a congenital brain malformation characterized by unilateral enlargement of the cerebral hemisphere. Hemimegalencephaly is an often isolated finding, but it has been described as an occasional feature of a large number of syndromes. We report a case of Hypomelanosis of Ito associated with hemimegalencephaly. Key words: Hypomelanosis of Ito, hemimegalencephaly, newborn