<p>Hydrocephalus remains a significant contributor to worldwide morbidity and mortality. Although factors like air pollution, noise, and shift work are suspected risk factors, causal inferences remain elusive. This study explored the effects of air pollution, noise, and shift work on hydrocephalus from a genetic perspective. In this study, Mendelian randomization analysis was used to assess the effects of air pollution (PM_2.5, PM_2.5–10, PM₁₀, NO₂, and NO_x), noise (day and night), and shift work on hydrocephalus. Genetic variants, strongly associated with air pollution, noise, and shift work, were extracted from genome-wide association studies (GWAS) and used as instrumental variables. GWAS data on hydrocephalus in European populations were obtained from FinnGen. The inverse variance weighted (IVW) method showed a potential causal relationship between PM_2.5 and Obstructive hydrocephalus (<i>OR</i>: 8.500 [1.136–63.595], <i>p</i> = 0.037) as a risk factor; Daytime noise (<i>OR</i>: 4235.704 [1.547–11,595,630.048], <i>p</i> = 0.039) and Evening noise (<i>OR</i>: 2457.341 [1.124–5,371,410.301], <i>p</i> = 0.047) with Post-traumatic hydrocephalus as a risk factor with a potential causal relationship. Our findings present genetic evidence underscoring the importance of enhancing residential environmental quality, specifically through the mitigation of air and noise pollution, as a potential strategy for reducing the risk of hydrocephalus. Subsequent studies are warranted to explore the impact of these exposures on disease progression and clinical outcomes in affected individuals.</p> Graphic Abstract <p></p>