Objective To summarize the clinicopathological features of fibro-adipose vascular anomaly (FAVA) in children, explore its diagnosis and treatment, improve the understanding of the disease by clinicians and pathologists, and reduce the rate of misdiagnosis. Methods The clinical data of 22 children with suspected FAVA admitted from July 2016 to February 2022 were retrospectively analyzed. According to the clinical data, the clinical characteristics were analyzed, and the key points of imaging and pathological diagnosis were summarized. Results Twenty-two patients (11 boys and 11 girls, with mean age of 7.22 years) met the clinical, radiological, and histopathological inclusion criteria for FAVA. The clinical presentation is a slow-growing mass with pain and/or contracture. Magnetic resonance imaging showed heterogeneous intramuscular, intermuscular or subcutaneous high and low signal mixed diffuse lesions with venous dilatation. Pathological findings included dense fibrous tissue in skeletal muscle, excessive fat, focal aggregation of blood vessels, lymphatics and lymphocytes and plasma cells, vascular hyperplasia, and skeletal muscle atrophy. In 22 cases, except for 2 cases of palliative resection and 1 case of local disengagement surgery, the remaining 19 cases underwent macroscopic complete resection. The follow-up period ranged from 2 months to 5 years and 10 months. Two children recurred, with a recurrence rate of 10.5%. The pain disappeared in all children except the recurrence cases.Conclusions Early surgery is recommended for vascular tumors/malformations that do not respond to repeated sclerotherapy and for those that cannot be clearly diagnosed clinically or radiographically. Postoperative pathology can confirm the diagnosis, and surgery can achieve the purpose of treatment.