Werdnig-Hoffmann disease refers to the severe infantile form of anterior horn cell degeneration. We report an association between Werdnig-Hoffmann disease and agyria-pachygyria. Examples of anterior horn cell disease with lesions in the central nervous system (notably thalamus and cerebellum) have been considered unusual "variants" of Werdnig-Hoffmann disease. This association between Werdnig-Hoffmann disease and agyria-pachygyria has, to our knowledge, never been described.