Ebstein anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced through the apex of the right ventricle. Giant congenital melanocytic nevi originate from melanocytes. It is usually seen after birth. Here we present the first neonate with prenatally diagnosed with Ebstein anomaly with giant hairy nevus in the literature.

Keywords: Ebstein anomaly, giant hairy nevus, neonate