Evans syndrome (ES) is a rare hematologic disorder characterized by the concomitant or sequential association of warm Auto-immune hemolytic anemia (AIHA) with Immune thrombocytopenia (ITP) and less frequently autoimmune neutropenia. Patients with combinations of autoimmune diseases carry a worse prognosis and are often difficult to treat as they are at risk of post-operative bleeding also the drugs that are routinely used in treatment carry a potential adverse reaction. Knowing this medical condition is paramount to providing safe and effective dental treatment and preventing undesirable events. This article aims to report a case of management of a patient with Evans syndrome, treatment of Evans syndrome, and suggests a guide for dental practitioners when treating patients with the condition.