Background: Pleuroparenchymal fibroelastosis is considered a rare form of interstitial lung disease. There are a growing number of case reports, however no large-scale studies detailing both clinical and histopathological features along with long term follow-up. An association with asbestos exposure has been described however this too has been limited to a few case reports. Case Report: A 73-year-old man with a history of interstitial lung disease and asbestos exposure presented with dyspnea with biopsy and imaging suggestive of pleuroparenchymal fibroelastosis. Despite steroid and anti-fibrotic therapy, the disease advanced rapidly causing the patient and family to pursue palliative care. Conclusion: Asbestos exposure is a significant risk factor for pleuroparenchymal fibroelastosis, a rare but increasingly recognized entity. Biopsy and image findings are the mainstay of diagnosis, pulmonary function tests are useful in prognostication. Treatment is supportive.