Goltz syndrome, also known as focal dermal hypoplasia syndrome, is a rare congenital mesoectodermal disorder. Two cases, which we experienced, showed erythematous, tan skin rashes and atrophic scars on the whole body, but there were some differences in clinical manifestations. Histopathologic findings in both cases showed diminution in the thickness of the dermis with subcutaneous fat extending upward to the epidermis. Therefore, we report a comparison of two cases of Goltz syndrome, especially with respect to clinical manifestations.