Ninety-four patients who had brachial plexus birth palsy were entered into a prospective study to evaluate the association between persistent palsy, age-related musculoskeletal deformity, and functional limitations. Of these patients, forty-two had either computerized tomography or magnetic resonance imaging to assess the presence and degree of incongruity of the glenohumeral joint, deformity of the humeral head, and hypoplasia of the glenoid as part of the preoperative planning for a reconstructive operation. Functional ability was rated with use of the classification of Mallet, on a scale of 1 to 5. The mean glenoscapular angle (the degree of retroversion of the glenoid) on the affected side was -25.7 degrees compared with -5.5 degrees on the unaffected side. Twenty-six (62 per cent) of the forty-two shoulders had evidence of posterior subluxation of the humeral head, with a mean of only 25 per cent (range, 0 to 50 per cent) of the head being intersected by the scapular line. Progressive deformity was found with increasing age (p < 0.001). The natural history of untreated brachial plexus birth palsy with residual weakness is progressive glenohumeral deformity due to persistent muscle imbalance. The status of the glenohumeral joint must be addressed when the choice between tendon transfer and humeral derotation osteotomy for reconstruction of the shoulder is considered for these patients.