Aims To analyze the factors affecting the prognosis of hepatoblastoma (HB) with lung metastasis in children. Methods The HB patients with lung metastases admitted to Beijing Tongren Hospital, Capital Medical University were collected. The clinical data, overall results, and prognostic factors were analyzed. Multivariate analysis was done by the Cox proportional hazards model for patients' prognosis. Results Finally, 98 HB patients (64 boys and 34 girls) with lung metastasis met the inclusion criteria, in which 64 patients had lung metastases at diagnosis (median age, 22.3 months) and 34 patients developed lung metastases while on treatment (median time, 6.5 months). The survival time and 5-year survival rate of patients with standard treatment were significantly longer than that of without standard treatment (P < .001). The survival time and 3-year survival rate had no difference between patients underwent lung metastasectomy and without lung metastasectomy (P = .099), between different diagnosis time of lung metastasis in HB patients (P = .37), between each histology type (P = .313), and different PRETEXT stage (P = .353). While the survival time and 3-year survival rate of patients with lung metastasis alone were significantly longer than that of patients with extrapulmonary involvement (P = .007). Multivariate Cox proportional hazards model revealed that the lung metastasis accompanied with extrapulmonary involvement was a risk factor affecting prognosis (HR = 0.460, 95% CI 0.239-0.888). Conclusions For HB children with lung metastatic, extrapulmonary involvement might be a high-risk factor of prognosis and standardized treatment with lung metastasectomy might prolong the survival time of them.