Exposures to toxic metals such as mercury have been suggested to be risk factors for amyotrophic lateral sclerosis (ALS). Human intake of mercury commonly occurs via consumption of seafood or from mercury-containing amalgam dental restorations (‘mercury fillings’). We therefore compared mercury exposures from these sources in 401 ALS and 452 non-ALS respondents, using an internationally-available online questionnaire that asked respondents how often they ate seafood and what their favourite types of seafoods were. Respondents were also asked to record numbers of current or former mercury fillings. ALS and non-ALS respondents did not differ in their frequency of seafood consumption or in monthly mercury intake from favourite seafoods. Both groups had similar numbers of current, as well as former, mercury fillings. In conclusion, this study found no evidence that mercury exposure from eating seafood, or from mercury dental fillings, was associated with the risk of developing ALS. Therefore, if mercury does play a role in the pathogenesis of ALS, other sources of exposure to mercury in the environment or workplace need to be considered. Alternatively, a susceptibility to mercury toxicity in ALS, such as genetic or epigenetic variations, multiple toxic metal interactions, or selenium deficiency, may be present.