Objective: To verify if specific foods and nutrients could be risk factors or protective factors for amyotrophic lateral sclerosis (ALS). Methods: Patients with newly diagnosed ALS from three Italian administrative regions were included. For each patient, a healthy control, matched for age (+/- 5 years), sex and administrative region of residence, was selected by a general practitioner. Cases and controls were interviewed by a trained investigator who filled a validated and reproducible food-frequency questionnaire. Daily intake of macronutrients, micronutrients, fatty acids, and total energy were estimated using an Italian food composition database. Results: Two hundred and twelve cases and 212 controls were included. A risk reduction was found for coffee and tea (odds ratios (OR)=0.29, 95% CI 0.14-0.60), whole bread (OR=0.55, 95% CI 0.31-0.99), raw vegetables (OR=0.25, 95% CI 0.13-0.52) and citrus fruits (OR=0.49, 95% CI 0.25-0.97). A risk increase was observed for red meat (OR=2.96, 95% CI 1.46-5.99) and pork and processed meat (OR=3.87, 95% CI 1.86-8.07). An increased risk was found for total protein (OR=2.96, 95% CI 1.08-8.10), animal protein (OR=2.91, 95% CI 1.33-6.38), sodium (OR=3.96, 95% CI 1.45-10.84), zinc (OR=2.78, 95% CI 1.01-7.83) and glutamic acid (OR=3.63, 95% CI 1.08-12.2). Conclusions: Some foods/nutrients may be risk factors and others protective factors for ALS.