We report the case of a 48-year-old woman diagnosed as having systemic lupus erythematosus who developed a neurological disorder that finally resulted in amyotrophic lateral sclerosis. Although neurological disturbances caused by systemic lupus erythematosus are protean, there are no reported cases of amyotrophic lateral sclerosis associating with lupus disease. This association and the suspected autoimmune mechanism of pathogenesis in amyotrophic lateral sclerosis suggest that this might not be a simple coincidence but perhaps a causal relationship.