ABSTRACT
Pulmonary capillary hemangiomatosis (PCH) is a rare disease usually associated with pulmonary hypertension which is classified in group I as per the World Health Organization (WHO) classification of pulmonary arterial hypertension (PAH). It is commonly presented as effort intolerance and hypoxia. Pulmonary capillary hemangiomatosis can present with ground-glass opacity on chest radiograph which can mimic interstitial lung disease. We need to consider PCH also in the differential diagnosis of interstitial shadows with these clinical presentations and should consider diagnostic evaluation for the same in case of unsatisfactory clinic-radiological response to treatment. We describe a case presented and treated as interstitial lung disease, which turned out to be PCH on lung biopsy. Interestingly, the case was not associated with any evidence of PAH.