Lipid pneumonia is pneumonia due to aspiration or inhalation of various oily or fatty substances. It can be divided into exogenous and endogenous depending on the source of the lipids. In endogenous lipid pneumonia (ELP), lipid accumulates in the intra alveoli as a result of obstruction, chronic lung infection/disease, or a lipid storage disorder. This study presents a case of a 47 year-old man with a history of smoking, surgically repaired ventricular septal defect, pulmonary stenosis, and no history of lipid intake. He complained of worsened exertional dyspnea and a chronic non-productive cough with no signs and symptoms of infection. The patient was diagnosed with idiopathic endogenous lipid pneumonia after excluding any inflammatory or systemic disease as a possible cause. Open lung biopsy showed lipid-laden macrophages. Corticosteroids were the mainstay therapy with no radiological improvement, eventually death occurred due to pulmonary embolism.

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