Congenital absence/hypoplasia/fusion of ribs are very rare anomalies and presentation varies from asymptomatic to life-threatening. Visibly evident cases are straightforward to diagnose but this is not always the case. Familiarity and awareness of these anomalies can help to diagnose cases with subtle signs and symptoms. Proper radiological investigations are vital for anatomical delineation. Absence or hypoplasia of the inferior ribs along with its attached muscles can cause ‘lung hernia’ that produces an unstable chest leading to paradoxical respiratory movements. Here, we present a case of hypoplastic and fused ribs in an infant, who presented with respiratory distress and created a diagnostic dilemma.