BACKGROUND Congenital hydrocephalus (CH) is a frequently encountered birth anomaly that can hinder long-term neurologic maturity and social well-being of affected children. This study was undertaken to assess quality of life (QOL) 10-15 years after surgical treatment for primary CH during infancy at a tertiary care hospital in a developing country. METHODS This retrospective cohort study included individuals who presented to Aga Khan University Hospital, Karachi, Pakistan, between 1995 and 2005 at <1 year old and underwent surgery for primary CH. The Hydrocephalus Outcome Questionnaire was used to assess outcomes with respect to QOL. RESULTS Of 118 patients, 90 patients participated in the study. Mean age at first admission was 6.2 months. Mean length of follow-up was 5.4 years. Of these, 28 patients had died after surgery. Shunt infection (P = 0.012) and delayed milestones (P = 0.003) were found to be statistically significant factors affecting mortality in the patients who died. The mean overall health score was 0.67 ± 0.30. Age <6 months at the time of first surgery was a poor predictor of overall health on the Hydrocephalus Outcome Questionnaire (P = 0.039). CONCLUSIONS In our analysis, we assessed the QOL associated with CH. We hope that these results will provide insight for future prospective work with the ultimate goal of improving long-term QOL in children with CH.