A retrospective record analysis of the last six years was done to determine the pattern of congenital malformations in the paediatrics department of Gondar College of Medical Sciences Hospital, North Gondar, Ethiopia. Of a total of 7489 admissions, 191 cases of congenital malformations were found, accounting for about 2.6% of the total admissions. The male to female ratio was 2.7:1. Cardiac malformations were seen more frequently (31%) followed by hernias (15.7%) and gastrointestinal malformations (14.8%). In only 45% of the cases were medical care sought in the first year of life. Out of 191 cases, 20 died in which the malformations were directly or indirectly accountable, giving a case fatality rate of around 10.5%. This study has highlighted the need for a community based, large scale prospective study to determine the incidence and contributory factors for congenital malformations.