A 51-year-old woman with a 5-1/2 year history of primary biliary cirrhosis (PBC) developed interstitial lung disease (ILD). The initial pulmonary infiltrate consisted of unilateral nodules mimicking neoplastic disease and later progressed to involve both lungs with a diffuse interstitial process. Tissue obtained by open lung biopsy was compatible with lymphoid interstitial pneumonia. The pulmonary infiltrates cleared spontaneously but recurred on two more occasions with subsequent resolution. This pulmonary disease activity appeared unrelated to the chemical activity of the PBC, which progressed to a fatal termination. The association of ILD, especially of a recurrent nature, with PBC is rare.