Bronchial variant of the upper lobe of the right lung, also called pulmonary isolation sign, is a congenital malformation that can be categorized into intralobar and extralobar types. Bronchiolar variant of the upper lobe of the right lung, also called pulmonary isolation sign, is a condition in which a portion of the lung tissue is separated from the normal lung tissue and develops separately during embryonic life; this portion of the lung tissue is not connected to the normal bronchial tubes and the blood supply to this portion of the lung tissue is also derived from an abnormal branch of the circulation. Bronchiolar variant of the upper lobe of the right lung is an abnormal development of lung tissue during embryonic period, which is a congenital malformation and can be divided into intralobar and extralobar types. Patients who suspect bronchiolar variant of the upper lobe of the right lung with cough, hemoptysis, dyspnea and other discomforts should seek prompt medical attention and active treatment.