Primary pericardial mesothelioma is a rare clinical entity; about 150 cases have been described in literature. It is a highly aggressive tumour with global survival under 6 months. We present a 44-year-old man with a primary pericardial mesothelioma who received treatment under clinical essay with carboplatinum and pemetrexed reaching tumoral response; consolidation radiotherapy was administered. Ten months after end of radiotherapy, tumoral progression was detected, and patient received second line of chemotherapy. The patient died 16 months after diagnosis. New cytotoxic drugs can improve the prognosis of this rare entity.