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Congenital pseudoarthrosis of the clavicle is a rare developmental condition characterized by a nonunion of the clavicular bone, typically presenting as a fibrous or cartilaginous tissue formation at the site of the defect. This condition arises during embryonic development, resulting in the incomplete formation or failure of the clavicle to unite properly. The exact etiology remains largely unclear, though several genetic and environmental factors may contribute to its occurrence, suggesting a potential multifactorial origin. The clavicle, a crucial bone that connects the arm to the body and provides structural support, can undergo abnormal development due to genetic mutations or disruptions during fetal development, leading to its incomplete formation. Clinically, congenital pseudoarthrosis of the clavicle can manifest with a noticeable deformity, altered shoulder mechanics, and potential complications such as pain or functional impairment. Patients may present with a visible difference in shoulder height or contour, and some may experience limited range of motion or instability in the shoulder girdle. The presence of a pseudoarthrosis can be discovered incidentally during imaging studies conducted for other issues or can be actively sought due to associated symptoms. Radiographically, an absence of normal bony continuity at the affected site is evident, often accompanied by an irregular, sclerotic appearance of the neighboring bone. Treatment options for congenital pseudoarthrosis of the clavicle are varied and may depend on the age of the patient, the severity of the condition, and associated symptoms. In asymptomatic individuals or those with minimal deformity, a conservative approach may be adopted, focusing on monitoring and physical therapy to maximize shoulder function. However, in cases where pain, significant deformity, or functional limitations are present, surgical intervention may be warranted. Procedures can include reconstructive surgery to promote bony union or stabilization techniques to enhance the shoulder's mechanical stability. Post-operative rehabilitation is crucial, emphasizing strengthening and range of motion exercises to ensure optimal recovery and functional outcomes. The overall prognosis for individuals with congenital pseudoarthrosis of the clavicle varies, largely depending on the extent of the deformity and the success of the chosen treatment modality. Although many individuals adapt well and maintain satisfactory shoulder function, others may experience ongoing challenges, necessitating long-term management strategies. Understanding congenital pseudoarthrosis of the clavicle necessitates a comprehensive approach involving careful assessment, targeted treatment, and ongoing evaluation to optimize patient outcomes and quality of life. Advancements in surgical techniques and a deeper understanding of the condition's underlying biology continue to shape management strategies, offering improved hope for patients suffering from this complex musculoskeletal anomaly.