Objective By analyzing and summarizing the clinical characteristics of pulmonary capillary hemangiomatosis (PCH), to enhance clinical physicians’ recognition, diagnosis and treatment capabilities for PCH. Methods Clinical data of two cases of PCH, who were diagnosed in Nanjing Tower Hospital, were reported retrospectively. Simultaneously, by using “Pulmonary Capillary Hemangiomatosis” as keywords, a total of 227 relevant articles was retrieved from domestic and international databases and 113 articles of case report were analyzed. Results Two cases of PCH patients were diagnosed eventually in our hospital, which were initially misdiagnosed as other diseases. After follow-up, the first case underwent lung transplantation four months after diagnosis, and the second case had been regularly taking medication to decrease pulmonary arterial pressure. Currently, two patients were in stable condition with pulmonary artery pressure significantly reduced. In literature review, 93 patients’ medical records were included in this study. We summarized the diagnostic methods, clinical manifestations, genetic testing, function check results, imaging characteristics, histopathology, treatment and prognosis of these patients. Conclusions PCH is very rare. The extremely low incidence rate and nonspecific clinical manifestations lead to high risk of misdiagnosis and underdiagnosis. Therefore, fully understanding and grasping its clinical characteristics are crucial for reducing misdiagnosis and underdiagnosis in the future.