We describe a woman with primary Sjögren's syndrome who presented with an acute pulmonary-renal syndrome resulting from cryoglobulinaemic vasculitis. Pulmonary manifestations of Sjögren's syndrome are relatively common, whereas overt pulmonary complications of cryoglobulinaemia are rare. Pulmonary haemorrhage is rare in either disorder. The combination of Sjögren's syndrome, cryoglobulinaemia, and acute pulmonary haemorrhage has not been previously reported.