We report a case of familial syringomyelia consisting of two sisters, one of whom had syringomyelia and Chiari type I malformation and the other, who had died 5 years earlier, had syringomyelia and Klippel-Feil syndrome. Although syringomyelia is uncommon and familial cases rare, they continue to be reported from time to time. Review of the literature certainly suggests that genetic factors play a part in the etiopathogenesis of syringomyelia related to abnormalities of the hindbrain structures, although the number of cases is too small for the exact nature of transmission be established.