Though a relatively large number of cases of congenital atresia of the bile ducts has been reported, attention has been restricted for the most part to the academic, if interesting, discussion of the anomaly at autopsy and to the possible etiologic factors involved. Little has been written on the management of these cases. Little has been said in reference to the worth of therapeutic procedure and the indication for surgical intervention. We report a case in which attention is focused on the problem of what to do for the child. We present the difficulties which confronted us in establishing our diagnosis and the perplexities which troubled us in planning our therapy. We shall, therefore, discuss in some detail the problems of differential diagnosis, but more especially do we wish to take up the question of the wisdom of surgical intervention on the basis of the underlying pathologic changes.