A Case Of Hemimegalencephaly With Hypomelanosis Of Ito

Hypomelanosis of Ito (Incontinentia pigmenti achromicans) is a rare phakomatosis characterised by hypopigmented patches or whorls along the (lines of Blaschko) with involvement of central nervous system,eyes and musculoskeletal structures. Very few reports of its association with hemimegalencephaly are reported. Reporting a 31 day female baby born for non consanguineous parents presented with refractory seizures. The child had a hypopigmented patch over left temporal region ,was clinically diagnosed as Hypomelanosis of Ito. MRI brain revealed the malformation. The case is reported for its rarity and its rarer association.