Sparganosis has a world wide distribution, but only a few patients have pulmonary involvement. The term sparganosis is defined as an infection by the larva of parasitic tapeworms of Spirometra species. We present here-in a patient, who was infected by this parasite and had pulmonary symptoms. The chest roentgenography revealed diffuse multiple nodular infiltration with cavitations. Bronchoscopy with a transbronchial lung biopsy was nondiagnostic. Finally, open lung biopsy was performed, and the histologic examination revealed plerocercoid larva of sparganum. The patient was treated with mebendazole 40 mg/kg/day for 6 months and his symptoms and pulmonary function improved. In the 5th year of follow-up, he presented with more progressive dyspnea and developed cor pulmonale, and finally died from pneumonia with sepsis. The objective of this report was to present a rare manifestation of sparganosis and it's clinical course. Currently, there is no known effective treatment for this disease.