Objective To improve the recognition of clinical features,image,and pathology of pulmonary lymphangiomyomatosis (LAM) and pneumoblastoma and enhance treatment at early stage.Methods A patient with pulmonary LAM and pneumoblastoma was analyzed including clinical data,image and pathological features,and responses of treatment,who was admited in 2013 March.Three articles and one review were searched from January 1967 to February 2014 in PubMed database with "lymphangiomyomatosis,cancer" as the search terms.A case report was searched from January 1982 to February 2014 in Wanfang database.Results The main symptoms of this patient who was female and 37 years old were cough,hemoptysis,and chest pain for three months.Though she had hamartomas of bilateral kidney,she had ectomy of hamartoma of right kidney,and biopsy indicated angiomyolipoma.Spontaneous pneumothorax was occured in past.Chest high-resolution CT indicated right lung malignant disease and pulmonary LAM.The serum vascular endothelial growth factor-D concentration of this patient was 1092.61 ng/L.Lung biopsy confirmed as pneumoblastoma:immunohistochemisty CD56 (+),Vim (+),Syn(+),CD99(+/-).Through the review of literature,there was no case of LAM combined with pneumoblastoma reported.Conclusions In recent decade,LAM is defined as a low-grade,destructive,metastasizing neoplasm with the molecule pathogenetic development of LAM.The patient with LAM and pneumoblastoma was first reported in domestic and international literature.However,it is controversial and unclear whether it belongs to carcinomatous change of LAM cell or it originally includes two malignant neoplasms.