Tricuspid valve stenosis and occlusion of superior vena cava are severe complications to Port-a-cath. In a child with SLE, symptoms started to develop about five to seven years after Port-a-cath insertion and cyclophosphamid injections. The patient developed hepatomegaly with abdominal and venous distension. Open heart surgery was necessary to remove the catheter. At operation it was found that the catheter was placed adjacent and through the tricuspid valve. The valve was severely stenosed with thrombus formation. The catheter and thrombus were removed, commissurotomy and bicuspidization of the valve and chordeal replacement performed to achieve an acceptable functional result. The superior vena cava was repaired with a pericardial patch. Retrospective analyses of the echocardiograms and chest x-rays show that the catheter was nearly related to the tricuspid valve and with the tip in the right ventricle. In such circumstances it is recommended with early withdrawal of the catheter, and in patients with immunological disease the indwelling time should be considered and limited.