Focal dermal hypoplasia (Goltz syndrome) is a rare congenital syndrome with suspected X-linked transmission that is characterized by a wide range of mesoectodermal defects. We describe a 39-year-old woman who had a peculiar phenotype and asymmetry of the body. Examination revealed atrophic erythematous and hyperpigmented linear streaks following Blaschko's lines, some of which were in a reticular or cribriform arrangement. Yellow, soft nodules caused by fat herniation were visible mainly in skin folds. The patient had multiple bone anomalies, including longitudinal striation of long bones (osteopathia striata). She had several ocular, dental, and kidney defects. Histopathologic examination showed a markedly thinned dermis that was replaced by adipose tissue.