We describe two brothers who developed chronic pulmonary disease in early childhood. Lung biopsies were diagnostic of lymphoid interstitial pneumonia (LIP). Familial LIP has not been previously reported, and the natural history is unknown. The elder brother experienced progressive respiratory disability and died 10 years after the onset of symptoms. The younger brother age 13, has been observed for 11 years and despite progression of pathological changes revealed in his second lung biopsy, he has had few symptoms and leads an active life. Current pulmonary function tests reveal decreased lung volumes, increased maximal expiratory flow rates and decreased lung compliance. Arterial PO2 is 75 mm Hg at rest and falls to 56 mm Hg with exercise. These findings, consistent with restrictive lung disease, contrast with the obstructive ventilatory pattern seen in some adult patients.