Bilateral pulmonary agenesis is a rare malformation with 13 cases previously reported. To our knowledge, none of these cases have been associated with hydrops fetalis. We report a 36 weeks, hydropic female infant (46XX) with bilateral pulmonary agenesis, unilateral microophthalmia, and bilateral renal dysplasia. These eye and pulmonary findings have now been associated several times and may constitute a new syndrome. Ultrasound examination before delivery showed polyhydramnios and fetal hydrops. At autopsy the heart was structurally normal, aside from absent pulmonary connections. The ductus arteriosus was partially closed. This was felt to be etiologic in the hydrops. As the source of the amniotic fluid here could not be urine or pulmonary secretions, direct fluid transfer from fetal vasculature or skin was the likely origin.