Background The clinical features and surgical outcomes of patients with Kaposiform hemangioendothelioma (KHE) is not fully clear. The purpose of this study is to better understand the therapeutic effect and hematological indices of surgical therapy for KHE. Procedure A retrospective study was conducted to review the medical documents of 43 patients with KHE who were treated with surgical resection at our hospital between February 2016 and November 2019. Multiple anatomical sites were involved. The curative effect was evaluated according to the tumor volume shrinkage, color shade, and blood examinations including platelet count, hemoglobin level, red blood cell number, clotting time and D-dimer concentration. Results For all cases underwent surgical treatment, the curative effects accounted for 100%. At the end of the treatment, 9 children were followed up for 6 months, and no evidence of the recurrence of KHE was not found in any case. The number of red blood cells and clotting time were maintained at normal levels during different stage of treatment. The concentrations of hemoglobin and fibrinogen and the average number of platelets increased significantly after surgical treatment. The concentrations of D-dimer were much higher than normal value during admission with a high variability, and significantly decreased after surgery treatment. Further analysis found that the changing trends of D-dimer was positively correlated with fibrinogen protein or platelet number, but not clotting time. Conclusions The surgical treatment method is highly effective and of great significance for KHE therapy in pediatric clinic.