Macrodystrophia lipomatosa is a unusual and bizarre congenital malformation of obscure etiology and pathogenesis and characterized by progressive overgrowth of all the mesenchymal elements of a digit or digits with a disproportionate increase in the fibroadipose tissues. Authors experienced four cases of macrodystrophia lipomatosa(three cases in the fingers and one case in the toe )and describe its radiologic and pathologic findings which differentiated this entity from other forms of macrodactyly. Radiologic findings in clude enlargement of the phalanges and soft tissue elements of the digit with a predominant involvement in distal part clinodactyly and radiolucencies in the soft tissue representing the dramatic overgrowth of fat.