This is a case report of a 53-year-old male with pulmonary alveolar microlithiasis. He was diagnosed as having pulmonary alveolar microlithiasis at the age of 50 from a chest X-ray that was one of several occasionally taken at a health examination. Chest X-rays taken at the ages of 6 and 13 years were retrospectively confirmed to have characteristic findings of pulmonary alveolar microlithiasis. His asymptomatic sister demonstrated a chest X-ray characteristic to pulmonary alveolar microlithiasis. Mutation of SLC34A2 gene was identified. He was referred to our hospital because of dyspnea on exertion and renal dysfunction. The latter was diagnosed 2 years ago and was caused by renal sclerosis resulting from hypertension. With the progression of both chronic respiratory failure and renal failure, he was put on a mechanical ventilation and peritoneal dialysis. Dialysis did not decelerate the progression of respiratory failure. To our best knowledge, this is the first case of pulmonary alveolar microlithiasis in which the effect of dialysis was evaluated.