Here we describe a statistically significant association between hypospadias and spina bifida. This association was identified through the Spanish Collaborative Study of Congenital Malformations (ECEMC), where the observed number of cases was greater (P << 0.0005) than expected just by chance among males. We also detected a pair of concordant twins who had monochorial monoamniotic placenta. To the best of our knowledge, this is the first report of this combination of defects, consequently it must be confirmed whether this statistic association is an entity.