This case presentation relates to a 21 year young male, cachectic in appearance, who presented with progressive shortness of breath, and dull pain on the lower part of the chest ongoing over three months. The patient received antitubercular drugs by local physician for suspected tuberculosis with no significant improvement. He was referred to our Respiratory Department for further evaluation of his chest pain and breathlessness. His X-ray showed moderate pleural effusion on the left side which confirmed by USG chest screening which showed multiple septation with thick parietal pleura and USG abdomen was normal. Moreover, the pleural effusion aspiration was performed and pleural fluid examination revealed exudative in character and thoracoscopic view of pleura was reddish with exudatives. Pleural biopsy histological examination showed mixed cell infiltrate with predominantly foamy macrophage along with plasma cells, lymphocytes and eosinophils with capillary proliferation. This is the first care report of xanthomatous pleuritis in the literature.