Terms used to describe congenital hypopigmentation and hyperpigmentation along the lines of Blaschko, including nevus depigmentosus, hypomelanosis of Ito, linear and whorled nevoid hypermelanosis, and cutis tricolor, are often confusing. Hypomelanosis of Ito, also known as incontinentia pigmenti achromians was first described by Ito in 1952. It is a kind of neurocutaneous disorder characterized by skin lesions along the Blaschko’s line. Approximately half of these patients have had logic, skeletal, and/or ocular abnormalities. Two years old female was visited to our clinic because of bizarre, reticulated, linear and whorl like hypopigmented lesions following the Blaschko line. The lesions presented at birth and developed without any inflammatory signs. There were no familial history of pigmentary disorder. She was delivered normally at term, and mental, bony and occular manifestations were not found. Histologic studies showed that the hypopigmented areas contained comparatively normal melanocytes and melanin. There were systemic anormalities, but based on clinical feature was suggestive of hypomelanosis of ito. This pattern of the cutaneous lesions suggests that the condition may result from the presence of two different cell populations as a result of mosaicism. Here, we report a case of hypomelanosis of ito resenting the characteristic hypopigmenta-tions along Blaschko's lines with systemic anormaly.