A13 years girl presented with history of sudden onset fits, altered sensorium and anuria for 2 days. There was also history of right sided weakness of the whole body. Past history revealed repeated episodes of similar complaints since early childhood. On the basis of history, physical examination and extensive investigations, patient was diagnosed as Upshaw-Schulman syndrome, a rare case of congenital Thrombotic Thrombocytopenic Purpura (TTP). She is now in remission and being maintained on twice weekly transfusions of Fresh Frozen Plasma (FFP).