Glenoid dysplasia is a developmental anomaly of the scapula that is characterized by a bony deficiency of the posteroinferior glenoid and the adjacent scapular neck. Glenoid dysplasia may occur as a primary isolated condition or in association with various syndromes. It is thought to be related to defective ossification of the inferior glenoid precartilage. Radiographs typically demonstrate bilateral, symmetric dysplasia of the scapular neck with a range of associated bony changes. Glenoid dysplasia has been associated with instability and premature glenohumeral arthritis, although the clinical presentation is highly variable. Symptoms are delayed or mitigated as a result of compensatory glenoid labral hyperplasia. The treatment of glenoid dysplasia remains challenging. Nonsurgical treatment is reasonably successful in younger patients, but premature degenerative changes frequently occur. Although favorable results can be obtained with the use of anatomic arthroplasty, continued subluxation and glenoid component failure can lead to unacceptable outcomes.