Background: Congenital unilateral absence of the pulmonary vein (UCAPV) is a rare entity with characteristic clinical and imaging findings. Despite its congenital nature, the radiographic findings and symptoms of UCAPV may not be recognized at birth and patients may present in childhood or early adulthood with findings that may mimic other diagnoses.
Methods: The evolution of imaging findings in UCAPV is presented through two cases, one of which demonstrates the progression of findings over several years.