OBJECTIVE The aim of this study was to assess myelomeningocele mortality, correlate these findings to lesion level, and investigate mortality evolution. METHODS From the population-based western Denmark myelomeningocele database, the authors extracted the records of 187 patients born between January 1, 1970, and July 1, 2015. Patients were categorized according to their most rostral lesion level into cervical, thoracic, lumbar, or sacral groups. Furthermore, patients were categorized based on their birth dates (1970–1979, 1980–1989, and 1990–2015). Mortality data was extrapolated from the university hospital's electronic charts, which are based on the Danish Civil Registration System, and compared according to mortality, lesion level, and date of birth. Data were also extracted from nationwide Danish registers. Additionally, the authors divided the patients according to date of birth before or after the advent of prenatal detection (2004), and compared mortality rates of these two groups. RESULTS A thoracic lesion level was associated with a significantly higher mortality rate (p = 0.01). Two patients had a cervical lesion and were alive at the end of follow-up. The mortality rate decreased over time, although not significantly for the subsequent time periods. Prenatal detection did not affect mortality. CONCLUSIONS The presented data suggest increased mortality with ascending lesion level in patients with myelomeningocele, except for patients with cervical lesions. The mortality rate improved over time, suggesting that modern treatment modalities improve survival in patients with myelomeningocele.