We report a case of pulmonary carcinosarcoma with jejunal metastasis. The lung is an exceptional localization for carcinosarcoma, a tumor with carcinomatous and sarcomatous components. These two components are closely related but well-defined morphologically and immunohistochemically. Risk of metastasis and local recurrence is high. Surgery is the treatment of choice for localized forms. Prognosis depends on the sarcomatous component which is usually sensitive to chemotherapy, with at least doxorubicin and ifosfamide. This rare case illustrates the potential for jejunal metastasis and complete response to chemotherapy, proven histologically at 33 months.