Hyperhomocysteinemia is a genetic disorder of metabolism and transport of amino acid, commonly present as a pro-coagulant state. Evan's syndrome is an autoimmune disorder with pancytopenia, a diagnosis of exclusion. The present report highlights the anesthetic management of a rare case, where both these clinical entities coexist. A 26-year-old male, a known case of hyperhomocyteinemia on medication for 4 years, came with a history of severe headache, blurring of vision and bleeding gums. Computerized tomography brain report showed subdural hematoma (SDH) of 16 mm with 9 mm right midline shift and on investigation had thrombocytopenia (5000 cells/cumm). Patient was diagnosed to have Evan's syndrome. Because he was refractory to the medical management, taken up for emergency splenectomy, followed by burr hole evacuation of SDH. Successful anesthetic management of the case is presented in this report.