Epstein syndrome is characterized by sensorineural hearing impairment, macro- thrombocytopenia without neutrophil inclusion bodies, and hereditary nephritis which can progress to end-stage renal disease (ESRD) in adolescence. The prognosis of Epstein's syndrome depends on the severity of the hematological disorders and renal involvement that can lead to ESRD at an early age. Thrombocytopenia in Epstein syndrome is not an absolute contraindication for major surgical procedures like arteriovenous fistula formation or kidney transplantation. There are no set guidelines for preoperative prophylaxis in a patient with this pathology.