The authors examined whether maternal dieting behaviors were associated with increased neural tube defect (NTD) risk among offspring, using population-based, case-control data. The analysis included 538 cases and 539 nonmalformed controls delivered from 1989 to 1991 in selected California counties, and exposures were assessed by in-person maternal interview. Among four reported dieting behaviors involving restricted food intake, diets to lose weight (odds ratio=2.1, 95% confidence interval: 1.1, 4.1), fasting diets (odds ratio=5.8, 95% confidence interval: 1.7, 20.0), and eating disorders (odds ratio=1.7, 95% confidence interval: 0.8, 3.6) were associated with increased NTD risk during the first trimester of pregnancy. Risk estimates for these behaviors during the 3 months before conception tended to be closer to 1. The fourth behavior, ‘other special diets,’ was not associated with increased NTD risk during either period. Women also reported whether they took diet pills, laxatives, or diuretics, engaged in binge eating, induced vomiting, or exercised excessively from the first 3 months before conception through the end of pregnancy. Only the intake of diuretics was associated with substantially increased NTD risk (odds ratio=2.7, 95% confidence interval: 0.7, 10.2). This study suggests that maternal dieting behaviors involving restricted food intake during the first trimester may be associated with increased NTD risk.