Wilms' tumour comprises 95% of all renal cancers among children less than 15 years of age. The purpose of this review is to examine the existing literature on perinatal and environmental risk factors for Wilms' tumour. A search for epidemiological studies that examined risk factors for Wilms' tumour was undertaken in Medline, LILACS, ISI Web of Science and Dissertation Abstracts. A total of 37 studies, including 14 cohort, 21 case-control and 2 case-cohort studies, were identified that examined environmental and perinatal risk factors. Most studies were from Western Europe and North America, and among case-control studies, 16 used randomly selected population-based controls. We observed a significantly increased risk of Wilms' tumour with maternal exposure to pesticides prior to the child's birth (OR = 1.37 [95% CI 1.09, 1.73]), high birthweight (OR = 1.36 [95% CI 1.12, 1.64]) and preterm birth (OR = 1.44 [95% CI 1.14, 1.81]), although the results regarding pesticide exposure may be subject to publication bias (Egger's test, P = 0.09). Further analyses to adjust for the heterogeneity in the results for high birthweight and preterm birth did not statistically change the significance of the results. Additionally, an increased though not statistically significant risk of Wilms' tumour was associated with maternal hypertension (OR = 1.30 [95% CI 0.99, 1.72]), and, compared with the first born, being a second or later birth was associated with a significantly decreased risk (OR = 0.82 [95% CI 0.71, 0.95]). This review suggests a role for several perinatal and environmental risk factors in the aetiology of Wilms' tumour.