We describe a patient with primary biliary cirrhosis (PBC) and secondary 
Sjogren's syndrome (SS) with pulmonary involvement who developed a cutaneous T 
cell lymphoma. The clinical course of secondary SS in PBC is thought to be less 
complicated than in progressive systemic scleroderma and SS. In contrast to 
secondary SS, the risk for developing non-Hodgkin's lymphoma is highly increased 
in patients with primary SS. Moreover, these lymphomas are usually of B cell 
origin. There are few reports of T cell lymphoma in primary SS. The occurrence 
of a T cell lymphoma in a patient with PBC and secondary SS indicates the 
necessity to investigate lymphoma in patients with secondary SS.