Misalignment of the pulmonary veins with congenital alveolar capillary dysplasia, although rare, has been reported as a cause of persistent pulmonary hypertension of the newborn. Reported cases have been mainly sporadic. Familial occurrence has been reported in only three instances. We present affected sibs with this condition. In addition to pulmonary abnormalities, urogenital abnormalities, including ureteric and urethral obstruction, seem to be common. Autosomal recessive inheritance is suggested.