Idiopathic interstitial pneumonias represent a diverse group of lung diseases with diffuse effects on the lung parenchyma. In 2002, the American Thoracic Society/European Respiratory Society consensus classification unified the descriptions of the different entities encompassed by idiopathic interstitial pneumonias. Despite this broad consensus there are still some entities without a clear definition and others, such as idiopathic bronchiolocentric interstitial pneumonia, that were only later described. We present the case and outcome of a woman diagnosed with idiopathic bronchiolocentric interstitial pneumonia by lung biopsy.