The radiological manifestation of frontometaphyseal dysplasia has been well elucidated in later childhood and adulthood, but it has not been thoroughly clarified in the neonate. Here we report the radiological features of a neonate with frontometaphyseal dysplasia. Most features, including twisted ribs, increased interpediculate distances of the lumbar spine, flared ilia, mildly flared and bowed long bones, and arachnodactyly with undermodeled short tubular bones, were reminiscent of those found in older children and adults. A previously undescribed abnormality was advanced ossification of the femoral and tibial epiphyses.