Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by progressive intra-alveolar calcification. We present a case of PAM with abnormal accumulation of 18F-fluorodeoxyglucose (FDG) in both lungs. A 55-year-old man was referred to our hospital for progressive dyspnea. He had been diagnosed with PAM 25 years earlier by transbronchial lung biopsy. High-resolution computed tomography revealed multiple dense calcifications with little aerated lung. Combined positron emission tomography and computed tomography using 18F-FDG (FDG-PET/CT) showed the abnormal accumulation of FDG in both lungs with a maximal standardized uptake value of 7.3. High FDG uptake was observed mainly in the lung regions showing sparing calcification. The patient died of respiratory failure a month later and an autopsy revealed no significant inflammatory changes in either lung. We suspect that the markedly enhanced pulmonary FDG uptake may have some relation to the pathophysiology of PAM.