Nineteen-year-old woman with mixed connective tissue disease developed Stevens-Johnson syndrome following treatment of arthritis using sulindac. Involvements of infectious and malignant diseases have been ruled out and sulindac has strongly been suspected as a causative agent for Stevens-Johnson syndrome. Ten out of 13 cases with Stevens-Johnson syndrome associated with non-steroidal anti-inflammatory drugs, sulindac has been administrated. Four cases also presented with severe liver disease. Patients who developed Stevens-Johnson syndrome following sulindac administration did not have apparent common clinical or laboratory findings which might be implicated for development of this severe side effects. Among the various non-steroidal anti-inflammatory drugs, safety of sulindac has widely been appreciated. However, occurrence of severe adverse events as reported here indicated that sulindac should be administrated as carefully as other non-steroidal anti-inflammatory drugs.