Objective:To analyze the clinical features and follow-up results of children with acute exogenous lipoid pneumonia (ELP), and to investigate the diagnosis strategy, treatment and risk factors of ELP.Methods:The clinical features, imaging manifestations, results of bronchoalveolar lavage fluid (BALF), treatment methods and prognosis of 41 ELP inpatients in Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from May 2013 to May 2018, were summarized and analyzed, and the related literature was reviewed.Results:(1) Among 41 patients, 29 cases were male and 12 cases were female.The age of onset ranged from 4 to 53 months.Most of them (35/41 cases, 85.4%) were less than 3 years old, and the majority were aged 1 to 2 years (23/41 cases, 56.1%). (2) The grease taken by children by accident included perfume oil (11 cases), white electric oil (7 cases), electric mosquito oil (5 cases), lubricating oil (5 cases), paraffin oil (4 cases), gasoline/diesel oil (3 cases), kerosene (2 cases), cod-liver oil (2 cases), sewing machine oil (1 case), and unclear oil (1 case). (3) The main clinical manifestations were cough (26/41 cases, 63.4%), shortness of breath (23/41 cases, 56.1%), fever (17/41 cases, 41.5%), cyanosis with hypoxemia (15/41 cases, 36.6%), wheezing (9/41 cases, 22.1%), respiratory distress (9/41 cases, 22.1%), pulmonary hemorrhage (6/41 cases, 14.7%), feeding difficulties and transient vomiting (4/41 cases, 9.8%). Some cases showed no symptoms (3/41 cases, 7.3%). (4) Chest X-ray mainly showed bilateral pulmonary exudative changes or pulmonary consolidation.The most common sign of high-resolution computerized tomography (HRCT) was local or extensive exudation, often accompanied by pulmonary consolidation (18/41 cases, 43.9%). The most common affected sites were bilateral lower lobes (8/41 cases, 19.5%), followed by right lower lobes (3/41 cases, 7.3%), right middle lobes (2/41 cases, 4.8%) and left lower lobes (2/41 cases, 4.8%). Other signs included alveolar changes, hyperventilation, ground glass changes, and nodule formation.(5) Seven children needed mechanical ventilation due to respiratory failure, and all patients underwent bronchoalveolar lavage (BAL). All patients recovered without death, with (11.67±4.90) days of hospitalization, and the hospitalization time of ELP patients with adenovirus infection was (19.25±5.93) days.Nine of them were lost to follow-up (8 cases were mild and 1 case was severe). The symptoms of the remaining children disappeared within 1 week to 1 month.The lung images of 21 cases returned to normal within 1 month after discharge, 7 cases returned to normal within 3 months after discharge, and 4 cases returned to normal after 6 months.Conclusion:The clinical manifestations and lung imaging of children with acute ELP have no specificity.Pulmonary imaging of severe patients shows multiple lobes involved, and consolidation and vacuolar changes may occur.Most patients have a favorable prognosis.Co-infection, especially adenovirus infection, may be a risk factor for acute ELP in children.BAL can play a role in both diagnosis and treatment, and its combination with local/systemic glucocorticoid therapy is effective for severe acute ELP.