A patient had primary ciliary dyskinesia with a complex cardiac malformation. As a child, she had benefited from a Fontan surgery to maintain a proper cardiac function. In such patients, whether it is safe to become pregnant is controversial. This case illustrates the possibility of carrying a pregnancy to term and providing a vaginal birth if a rigorous preconception consultation is performed to ensure care by a multidisciplinary specialized team, and the patient is properly informed of the risks.