Pseudomesotheliomatous adenocarcinoma of the lung is a rare entity, clinically and pathologically mimicking diffuse malignant pleural mesothelioma. We report a case of pseudomesotheliomatous adenocarcinoma consisting of two distinct histopathologic components with marked differences in morphological appearance and immunohistochemical staining pattern: a small subpleural nodule consisting of adenocarcinoma and undifferentiated carcinoma with rhabdoid features, the latter extending to the visceral pleura in a diffuse mesothelioma-like growth pattern. The adenocarcinomatous component showed strong reactivity with various epithelial markers, but no reactivity with vimentin. The undifferentiated component showed strong reactivity for vimentin, but no reactivity for epithelial markers. Both components showed no reactivity with CEA, Ber Ep4, MOC-31, B72.3, cytokeratin 5/6, calretinin and thrombomodulin. Metastatic lesions in the duodenum and the periumbilical skin consisted entirely of undifferentiated carcinoma with rhabdoid features. Fluorescence in situ hybridisation (FISH) was performed for chromosomes 4, 7, 20, and 22. FISH results showed polysomy 7 in both components. The diagnosis of pseudomesotheliomatous adenocarcinoma in this case was based on the finding of the subpleural nodule. We conclude that in cases of undifferentiated malignant pleural tumors with cells of rhabdoid phenotype, the possibility of peripheral pulmonary adenocarcinoma dedifferentiating into a more aggressive phenotype should be considered.