A case of congenital generalized fibromatosis in a black male infant with involvement of lungs, subcutaneous tissue, pancreas, adrenal, lymph nodes, and bone is described. Fifteen prior cases with pulmonary involvement are reviewed, emphasizing the poor prognosis associated with pulmonary lesions. This case demonstrated, in addition to previously described histologic features, broad bands of fibroblastic proliferation in the interlobular septa producing retraction of the visceral pleura. Possible etiologic factors are discussed, with suggestions for additional studies in future cases.