Background: Progressive bulbar paralysis of childhood is characterized by progressive cranial nerve palsies especially bilateral facial weakness, swallowing difficulties and bilateral ptosis. Fazio-Londe disease (OMIM 211500) is usually classified with spinal muscular atrophies of autosomal recessive or dominant inheritance. Resembling diseases are Vialetta-van Laere syndrome (OMIM 211530) and Madras-type motor neuron disease. Diagnosis consists of the clinical features, electromyograhic and histologic findings and the exclusion of other causes of progressive bulbar paresis.