This article deals with a young school boy born in 1971 with a tumor in the palm of his right hand. We have considered this a juvenile aponeurotic fibroma. This tumor has recurred several times, leading to unavoidable amputation of the right wrist and hand. Seven years after the initial diagnosis, the axillary area became involved, then the pleura and lung. Such an unusual process leads to reconsidering the diagnosis and to regard this case as an intermediate form between Keasbey's juvenile aponeurotic fibroma and generalized fibromatosis.
