The authors report 4 cases of urinary tract malakoplakia. This rare disease of unknown etiopathogenesis can present with a benign character in the bladder, but when the upper urinary tract is affected, loss of renal function can occur. Treatment aims to control the primary infection, as well as enhance intracellular bactericidal activity, which seems to be compromised in these cases. Cytoreductive surgery may be indicated when this treatment fails.