Background: The spectrum of CNS congenital anomalies varies from one geographical location to another across the globe. This essentially can be solitary or multiple and can be cranial, spinal, combination of both cranial and spinal or others. The understanding of their distribution in each location could determine local workforce and facility needs and as well guide in the areas of sub specialization. Objective: The aim of this study is to document the pattern of CNS congenital anomalies cases that had surgical intervention, at the Usmanu Danfodiyo University Teaching Hospital, Sokoto. Methods: A cross-sectional, retrospective hospital based study from January, 2021 to December, 2021 of all patients with CNS congenital anomalies who had surgical intervention at the Neurosurgery Department, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. Results: A total of 49 cases presented to our facility and had surgical interventions for central nervous system congenital anomaly. Of those 49 cases with CNS Congenital anomalies, there were 25 (51.0%) males and 24(48.98%) females, with a male-to-female ratio of 1.04: 1. The median age was 185 days, with age range of 2– 364days. Overall, the most commonly diagnosed neurosurgical CNS congenital anomalies in order of decreasing frequency were Congenital hydrocephalus 20 (40.82%), Myelomeningocele 14 (28.57%), Encephalocele 10 (20.41%), combination of Hydrocephalus associated with myelomeningocele 8(16.33%), Meningocele 3(6.12%), Lipomeningocele 1( 2.00%), Dandy walker malformation 1( 2.00%) , and Dandy variant1(2.00%) . Conclusions: NTDs (Myelomeningocele, Meningocele and lipomeningocele) were the most common CNS anomalies (55.10%). However, congenital hydrocephalus, Myelomeningocele, Encephalocele, Hydrocephalus + MM, and Meningocele are common in our environment in decreasing frequency. Though, Lipomeningocele, Dandy walker malformation and dandy variant are relatively rare. Understanding of this distribution may guide service expansion and workforce needs, inspire sub specialization as well as direct research and government policy in this community.