Background: Published data on congenital musculoskeletal anomaly in subsaharan Africa are few.Aim: To review the pattern of congenital musculoskeletal abnormalities seen at the Obafemi Awolowo University Teaching Hospitals' Complex (O.A.U.T.H.C) Ile-Ife.Materials and Methods: The clinical records of patients who presented to the O.A.U.T.H.C, Ile-Ife, with congenital musculoskeletal abnormalities over 7 years (July 2000 to June 2007) were reviewed. Comprising patients delivered in the hospital, referred patients seen in the Orthopaedic clinics and those admitted to the hospital Orthopaedic, Paediatric and Postnatal wards. The following data were extracted; age, gender, diagnoses, description of the abnormality and the presence of associated congenital deformities. The side of the body involved and bilaterality were noted.Result: Over this period, 123 patients met the criteria. Eighty one were males while 42 were females with a M:F= 2:1 Congenital talipes equinovarus(CTEV) deformity was the most common congenital musculoskeletal anomaly in 64(52.0%) patients. Others were Polydactyly 16 (13.0%) and syndactyly 11(8.9%). Other common congenital orthopaedic anomalies encountered include: Arthrogryposis multiplex congenital 7(5.7%), congenital knee dislocation 6(4.9%), Calcaneovalgus deformity 5(4.1%) and absent radius 5(4.1%). Among the patients with clubfoot, there was a predominance of bilateral involvement of both foot with 63.4% of the idiopathic variety showing bilaterality and 60.9% of the non-idiopathic variety having bilateral involvement. The idiopathic Variety constituted 64.1% of the club foot deformities. Spina bifida cystica was the most common associated deformity among the patients with nonidiopathic clubfoot.Conclusion: Congenital talipes equinovarus was the most common congenital Musculoskeletal anomaly seen at the Obafemi Awolowo University Teaching Hospital Ile-Ife, with a high incidence of bilaterality. Spina bifida cystica was the most associated anomaly in the non-idiopathic club foot in this centre.Keywords: Congenital, Musculoskeletal, anomaly, Tertiary, Referral, Centre