Rituximab is a chimeric monoclonal antibody that specifically targets the CD20 molecule on the B cell surface. Although rituximab was originally introduced for the treatment of lymphoid neoplasms such as non-Hodgkin’s lymphoma (NHL) and chronic lymphocytic leukemia (CLL), it is now emerging as an effective and relatively safe therapeutic option for the patients with refractory immune thrombocytopenic purpura (ITP). We report here on a case of life-threatening toxic epidermal necrolysis (TEN) that was related with the use of rituximab in a patient with refractory ITP. The patient developed extensive erythematous papules and bullous lesions on his whole body associated with fever and visual disturbance during the second cycle of rituximab. The rituximab was discontinued and high dose intravenous immunoglobuline and steroid were administrated. Four weeks later, he fully recovered without any sequelae. A review of the literature reveals this to be the first reported case of TEN associated