Congenital pseudarthrosis of the clavicle is a rare entity of unknown aetiology with predominance of the right side. Our therapeutic concept is discussed with special reference to surgical therapy, histopathological findings and the most recent literature. Two girls and one boy, aged 4, 6, and 8 years, presenting with congenital pseudarthrosis of the clavicle were surgically treated between 1994 and 2000. A resection of the pseudarthrosis and internal fixation with a small reconstruction plate was performed. A bone graft from the iliac crest was used for restoration of clavicular length. Histological examination revealed a false joint with the ends of the clavicle covered by hyaline cartilage. The patients showed radiographic healing after 12 weeks. At follow-up (mean 44 months), all patients showed excellent clinical and radiological results without functional impairment. The clinical features and histological examination of the resected pseudarthroses clearly proved the diagnosis of a true congenital pseudarthrosis of the clavicle. According to our clinical and radiological results and considering the recent literature, we recommend surgical therapy with resection, bone grafting, and osteosynthesis with a reconstruction plate around the age of 4 - 6 years.