iarjmcrIARJMCRIARJMCR2709-3220https://doi.org/10.47310/iarjmcr.2023.v04i02.003Pulmonary alveolar microlithiasis- A rare cause of Pulmonary Arterial Hypertension- a case report and review of literatureAnkitKumarNaveenYadavJayantGroverYUdayAnilMenonDMBackground: Dyspnoea is the common manifestation of Pulmonary hypertension (PH). Pulmonary alveolar microlithiasis is one of the rare causes of PH with a handful of case reports in the literature.  Case Presentation: A 59 years old male presented with complaints of progressive dyspnoea of 06 months and dry cough of 01-month duration. He had clinically he had tachypnoea, tachycardia, hypoxia and Grade III clubbing and bilateral crackles in both lower lung fields. Evaluation he had Sandpaper like calcifications and black pleura sign on imaging suggestive of PH due to pulmonary alveolar microlithiasis and restrictive pattern on spirometry for which he was managed conservatively to which he had symptomatic relief. He is on follow up and planned for investigational therapy in case of deterioration of lung functions.  Conclusion: Pulmonary alveolar microlithiasis is a rare genetic disorder leading to pulmonary hypertension. Our case is peculiar as it presented late in the 6th decade of life with usual clinical features. He is under closed follow up for deterioration and starting investigational therapy.