A case of unilateral Sprengel's deformity with an associated omovertebral bone is presented. A review of proposed mechanisms of development is included in the discussion of this rare anomaly. The condition is largely one of cosmetic deformity rather than signs and symptoms. The significance of identifying it's presence lies in uncovering the many less obvious, but more potentially serious, anomalies that may be associated with it. Conservative management is discussed.
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