The aim of this study was to determine the incidence of congenital malforma
tions in a complete cohort of children born after intracytoplasmic sperm in
jection (ICSI), The medical records were retrieved for 1139 infants, 736 si
ngletons, 200 sets of twins and one set of triplets. The total number of in
fants with an identified anomaly was 87 (7.6%), 40 of which were minor The
incidence of malformations in children born after ICSI was also compared wi
th all births in Sweden using data from the Swedish Medical Birth Registry
and the Registry of Congenital Malformations. For ICSI children, the odds r
atio (OR) for having any major or minor malformation was 1.75 [95% confiden
ce interval (CI) 1.19-2.58] after stratification for delivery hospital, yea
r of birth and maternal age. If stratification for singletons/twins was als
o done, the OR was reduced to 1.19 (95% CI 0.79-1.81). The increased rate o
f congenital malformations is thus mainly a result of a high rate of multip
le births. The only specific malformation which was found to occur in exces
s in children born after ICSI was hypospadias (relative risk 3.0, exact 95%
CI 1.09-6.50) which mag be related to paternal subfertility.