Background: Congenital disorders are structural, metabolic, behavioral and functional disorders that are present at birth. Their manifestations are protean ranging from mild anomalies to lifeâÂÂthreatening conditions. Aim: The objectives of this study were to describe the congenital anomalies in children seen at Federal Medical Center, Bida over a 12 month period, determine possible factors associated with these anomalies; and their short term outcome. Subjects and Methods: Children with clinically recognized congenital malformations were recruited consecutively over a 12 month period and socioâÂÂdemographic, etiologic and other relevant clinical data were obtained. A detailed examination was also performed and abnormalities documented. The data was analyzed using EpiâÂÂinfo version 6 (Atlanta, USA). The ChiâÂÂsquare was used to identify significant differences for categorical variables. MidâÂÂP and Fisher’s exact tests were utilized as appropriate. A P < 0.05 was considered to be significant. Results: A total of 46 children with congenital anomalies were seen during the study period, all which were recruited into the study. The hospital based prevalence amongst neonates was 111/1000 neonates. The most common system affected was the digestive system (50.0%) followed by the central nervous system and head and neck anomalies. There was no significant difference in distribution of anomalies amongst the various ethnic groups. About 22% of families were consanguineous, all being first cousins and 8.7% of mothers were greater than 35 years of age. The case fatality rate for congenital malformations was 2.2%, while 60.9% were referred to other hospitals for further care. Conclusion: The study has demonstrated a wide variety of congenital anomalies in Bida, NorthâÂÂCentral Nigeria with the digestive system anomalies being the most frequent. The findings of this study strengthen the need for empowerment of the institution in appropriate management of these disorders.