A 3-month-old boy underwent flexible bronchoscopy with bronchography for evaluation of airway compression associated with absent pulmonary valve syndrome. This showed a blind-ending bronchus, arising from the medial aspect of the right main bronchus opposite the origin of the right upper lobe bronchus (black arrow) (Fig. 1). There was extrinsic compression of the carina (white arrow) due to enlargement of the pulmonary arteries. The finding was confirmed by contrast-enhanced CT (arrow) (Fig. 2).

Fig. 1
figure 1

Bronchography

Fig. 2
figure 2

Contrast-enhanced CT

Bronchus cardiacus accessorius dexter is a rare anomaly, detected at less than 0.1% of adult bronchoscopies [1, 2]. It may coexist with other anomalies, as in this patient, but is rarely symptomatic [1, 2].