Objective　To summarize the clinical characteristics of neuroblastoma (NB) with refractory diarrhea as the main symptom in children. Method　The clinical data of neuroblastoma with refractory diarrhea as the main symptoms in 2 children were retrospectively analyzed and the literature was reviewed. Results　Both cases of neuroblastoma were female and over one year old and suffered from refractory diarrhea and hypokalemia. In case 1 neuroblastoma was confirmed by postoperative pathology and recurrent diarrhea occurred during chemotherapy. In case 2 onset symptom was diarrhea which was also most obvious symptom. She had electrolyte disturbance, abdominal distension and weight loss. Palpable abdominal mass was observed in the child. Neuroblastoma was diagnosed by imaging and pathological examination. Both cases were ganglion cell type NB, and their symptoms were relieved after surgery combined with chemotherapy. Both survived disease free. Conclusion　Neuroblastoma with refractory diarrhea as the main symptom is rare. There is no standard diagnosis and treatment. The combination of surgery and chemotherapy is effective.