A report is presented on a nine year old girl in whom a Spitz-Holter valve had to be implanted after operation on a meningomyelocele while a neonate. Because of a macrohematuria and a hypochromic anemia, she was admitted as an inpatient. In addition, there was a pyuria with bilateral hydronephrosis. The clinical picture was initially misinterpreted as hemorrhagic cystitis with ascending pyelonephritis in neurogenic bladder. Only the reduction of serum complement suggested the presence of a shunt nephritis. The diagnosis was verified by kidney biopsy. After removal of the infected valve system, there was a prompt normalization of all laboratory parameters. Besides description of the case history, above all the diagnostic problems of this rare syndrome, which is nevertheless of practical importance, are dealth with.