CASE PRESENTATION: A 40 year male presented with dyspnoea, cough and fever of 4 month duration, with history of frequent respiratory infection in child hood. On examination chest was asymmetric as there was flattening of infraclavicular area, decreased breathing movement, chest expansion &drooping of shoulder in right side. Trachea was shifted to right, Breath sound was absent in the right side with expiratory wheezes in left. Percussion note was dull on right and hyper resonance in left. X RAY CHEST showing homogenous opacity on right&compensatory emphysema on left side. CECT THORAX showing complete absence of right main bronchus with no visible aerated right lung, associated with marked mediastinal shift and cardiac rotation and compensatory hyper expansion with anterior herniation of the left lung. Right pulmonary artery and vein was absent. BRONCHOSCOPY also confirm absence of right main bronchus. PFT showed obstructive pattern and 2D ECHO CARDIOGRAPHY was normal. In Musculoskeletal examination there was low hair line with short neck, scoliosis with convexity to left and elevation of right scapula (sprengel’s deformity) with X RAY AND CECT cervical spine showing absent C1 and C6 cervical vertebrae with partial bony fusion involving right part of C7-T1 vertebral body suggestive of klippel-feil syndrome3.