We reviewed the recent advances in syringomelia based on nationwide survey on the epidemiology of syringomyelia in Japan. The estimated prevalence of ambulatory syringomyelia patients in Japan was 1.94 per 100,000. The proportion of asymptomatic syringomyelia patients was 22.7%. Chiari type I malformations and idiopathic syringomyelia were the first and second most common etiologies. Approximately 70% patients were performed foramen magnum decompression (FMD) with dural patch grafting and/or dural dissection. Several pedigrees with familial syringomyelia were observed. The progress of genetic analysis study is expected.