Background <p>Spinal dysraphism is a congenital condition requiring lifelong management to prevent long-term complications. Our institution is a referral center for congenital malformations in the Indian Ocean, particularly serving Réunion Island, Mayotte, Madagascar, and the Comoros. The aim of this study was to describe a cohort of children with spinal dysraphism followed at our center.</p> Methods <p>This retrospective cohort study included consecutive patients diagnosed with spinal dysraphism who were born between January 1, 2006 and December 31, 2022, and followed at our institution. Data were extracted from electronic records and covered the antenatal period, delivery, initial management and follow-up.</p> Results <p>Over the 17 years of the study period, 121 patients were included. The annual mean incidence was 2.1/10,000 live births and remained stable throughout the study period. Among the 121 patients, 82 had a prenatal diagnosis, and 69 underwent a proactive management. Proactively managed patients originated from Réunion Island (<i>n</i> = 41), Mayotte (<i>n</i> = 12), the Comoros (<i>n</i> = 12) and Madagascar (<i>n</i> = 3). Surgical closure was performed in all patients with open defects, and a ventricular shunt was used in half of the patients. The median follow-up duration was 4.2 years. The mortality rate was 4.3%, and 42% of patients were lost to follow-up. At least one clinical review was conducted by a neurosurgeon in 85% of cases, a urologist in 71%, or an orthopedic specialist in 49%. Neurodevelopmental delay was observed in all patients who required a shunt placement. Ambulation was achieved in 39% of the patients.</p> Conclusions <p>The incidence of spinal dysraphism remained stable over time in our population. The monitoring of patients was not optimal, but the remote origin of some of our patients must be taken into account. Further studies should be performed to evaluate continence and long-term complications.</p>