Epstein syndrome constitutes macrothrombocytopenia without neutrophil inclusion bodies along with deafness and renal failure. A diagnosis of Epstein syndrome was made in a 17 year-old-male patient with macrothrombopathic thrombocytopenia, renal failure and sensorineural hearing loss. Our patient is unique as he presented with rapidly progressive renal failure and developed chronic kidney disease in second decade of life with no symptomatic hearing loss or bleeding tendency. Epstein syndrome needs to be differentiated from Alport syndrome which is more common disease with similar clinical presentation.