Pena Shokeir is a primary motor neuropathy associated with the absence of anterior horn cells in the spinal cord and diffuse muscle atrophy. Muscles involved include those of the respiratory system, and underdevelopment of the lungs is evident. There are at least two forms of Pena- Shokeir; however, some researchers have identified five subtypes. Due to the variations, Hagerman, Willemse, van Ketel, Barth, and Lindhout (1987) suggested that Pena-Shokeir syndrome not be considered as a unique syndrome but rather a phenotype of fetal akinesia.