Heparin-induced thrombocytopenia (HIT) is the most important immunological drug reaction that patients face today. Being unfractionated heparin the standard anticoagulation used in haemodialysis, acute or chronic uremic patients starting haemodialysis are at risk of developing HIT. Through the accurate description of two patients, one with chronic and the other with acute uraemia, who developed this complication at the start of haemodialysis, we compare the distinct clinical problems of haemodialysis-related HIT with the general clinical features of HIT. We report the occurrence of repeated clotting of both dialysers and catheters, as well as thrombosis of the central veins where the catheters are placed and of the fistulas. We also report an accurate review of the literature on haemodialysis-related HIT. We have observed that HIT seems to be particularly rare in haemodialysis patients. Since newly treated haemodialysis patients are at risk of developing HIT, and most of the studies were made on long-term chronic haemodialysis patients, we assume that the syndrome is poorly documented. Our own experience on 37 haemodialysis patients who developed HIT is reported by focusing on both the clinical presentation of HIT as well as the long-term follow up of the patients. We present some considerations on the treatment options of acute HIT in uremic patients as well as on the problem of heparin re-exposure subsequent to the HIT episode, a very prominent problem in chronic haemodialysis patient.