AbstractEvans syndrome is an unusual condition characterized by simultaneous or sequential onset of autoimmune hemolytic anemia and immunothrombocytopenic purpura with a positive Direct Coomb’s Test, in the absence of a known underlying cause. A defect in humoral and cell-mediated immunity is considered to be the most likely cause of Evans syndrome. A positive Direct Coomb’s Test confirms the diagnosis. The disease follows a chronic relapsing course, and is associated with considerable morbidity and mortality. There have been very few reports of Evans syndrome from the Indian subcontinent. We report a 30-year-old woman with Evans syndrome who presented with life threatening autoimmune hemolytic anemia and thrombocytopenia. We will be discussing the pathogenesis, clinical presentation and treatment results of Evans syndrome.