A 55-year-old man was transferred to our hospital with unilateral lung lesions, a persistent fever and vague chest pain with arthralgia lasting for three months. He had been treated for end-stage renal disease with hemodialysis for 15 years and had a medical history of recurrent subcutaneous calciphylaxis due to secondary hyperparathyroidism. Transbronchial biopsied specimens demonstrated metastatic pulmonary calcification, and a bone marrow biopsy showed Philadelphia chromosome-positive acute lymphoblastic leukemia. Although metastatic calcification often lacks specific symptoms, the lungs is a primary site for deposition. This is the first report of unilateral metastatic pulmonary calcification associated with secondary hyperparathyroidism.