The nosology of scapuloperoneal syndrome remains controversial. It may be only a stage in the development of facioscapulohumeral dystrophy. The aim of this investigation was to re-establish the clues for distinguishing between scapuloperoneal syndrome and facioscapulohumeral muscular dystrophy. Twelve patients with a scapuloperoneal syndrome were investigated and compared with seventeen patients with facioscapulohumeral dystrophy. The distribution of weakness for both groups of patients was quite different. The electromyographic examination of patients with scapuloperoneal syndrome revealed typical mixed myogenic and neurogenic features and peroneal nerves involvement. The electromyographic findings in patients with facioscapulohumeral muscular dystrophy were typical for myogenic involvement. In conclusion we can suppose that the scapuloperoneal muscular atrophy is not a stage in the development of facioscapulohumeral dystrophy. It is a separate nosological entity with typical clinical and electromyographic findings.