Jo-1 syndrome is an autoimmune disease with autoantibodies against the histidyl tRNA synthetase. Characteristic clinical findings include inflammatory myopathy and interstitial lung disease. We present the first case of a patient with Jo-1 syndrome (positive Jo-1 autoantibodies, myositis, interstitial alveolitis) who developed Hodgkin's lymphoma of nodular-sclerosing type. Thus, patients with Jo-1 syndrome and immunosuppressive therapy similar to other patients with autoimmune disease are at risk to develop lymphomas and should therefore be monitored carefully.