In a retrospective study of 1224 transthoracic echocardiograms performed between January 2011 and December 2013, we evaluated the spectrum of congenital heart disease (CHD) diagnosed at a tertiary referral centre in Maiduguri, north-eastern Nigeria. Diagnosis of CHD was made in 88 (8.3%) subjects, comprising 23 (26.1%) adults and 65 (73.9%) aged less than 18 years. Forty six (52.3%) of those with CHD were females, while 42 (47.7%) were males. The frequencies of the CHD in decreasing order were: ventricular septal defect 23 (26.1%), tetralogy of Fallot (TOF) 14 (15.9%) and atrial septal defect (ASD) and atrioventricular septal defect (AVSD) were 11 (12.5%) each. One of the patients with AVSD had Ellis Van Creveldt syndrome. Six (6.8%) cases of patent ductus arteriosus (PDA) were diagnosed in those younger than 18 years, while all the 5 (5.7%) cases of Ebstein's anomaly were diagnosed in adults. There were 6 (6.8%) cases of Eisenmenger syndrome involving three cases of AVSD, one case of ASD and two cases of Ebstein's anomaly. Timely definitive cares for these patients are still lacking in Nigeria and many areas of sub-Saharan Africa. We recommend sensitization of all relevant clinicians to actively look for congenital heart defects. Pulse oximetry and postnatal echocardiographic new-born screening which were previously validated should be implemented at secondary and tertiary levels, and efforts should be made towards providing the needed care for patients with CHD.