Neu-Laxova Syndrome 1 (NLS1) is an extremely rare lethal congenital abnormality. This disorder is characterized by intrauterine growth retardation, distinctive craniofacial abnormalities (microcephaly, hypertelorism, prominent eyes, absent eyelids, wide nasal bridge, retrognathia, and cleft lips), flexion contractures, hypoplastic digits showing syndactyly, edema, generalized or localized to the face and scalp, hyperkeratosis, ichthyosis, and polyhydramnios. Neurological abnormalities include lissencephaly, dysgenesis of the corpus callosum, and cerebellar hypoplasia. Other abnormalities that may be present include rocker-bottom feet, abnormal external genitalia (especially in males), spina bifida, cryptoorchidism, cataracts, shallow orbital cavities, kyphosis, and webbing of the knee and elbow joints. Very few cases of NLS1 have been reported worldwide, and the condition seems not to show any sexual or ethnic preference.