We report a case of pseudotumoral, nodular, parenchymatous, primary pulmonary amyloidosis in a male patient under 50 years of age. The amyloidosis spread step by step, very progressively, over 12 years before Marie's disease developed. The pre-operative diagnosis was made by electron microscopy of an intrabronchial growth biopsy. Right pleuro-pneumonectomy was performed, resulting in regression of Marie's disease within 3 months. There was partial factor X deficiency. Four years after surgery, nodules have appeared in the left upper lobe, suggesting a recurrence.