Objective To investigate the clinical characteristics and prognosis of primary nephrotic syndrome (PNS) complicated with adrenal crisis (AC) in children. Methods Clinical data of 15 patients with primary nephrotic syndrome complicated with adrenal crisis in the department of Pediatrics, Boai Hospital, Zhongshan City from January 2010 to March 2022 were retrospectively analyzed. Results The average age of the patients was 6.25±1.81 years old (4.75-12 years old), including 9 males and 6 females. The course of disease was 2.74±1.33 years (1-6 years). Inducement: Glucocorticoid dose reduction or withdrawal in 8 cases, respiratory tract infections in 5 cases, and no obvious triggers in 2 cases. Clinical manifestations: Gastrointestinal symptoms such as abdominal pain, nausea, and vomiting were found in 11 cases, circulatory failure manifestations such as low blood pressure and oliguria in 7 cases, hyponatremia in 8 cases, hyperkalemia in 4 cases, and hypoglycaemia in 2 cases, All the children were accompanied by neurological symptoms of varying degrees (such as dizziness, debilitation, malaise or irritability, etc.). Serum cortisol: Random serum cortisol level of 9 children with digestive tract or early shock symptoms was 37.6±15.7 nmol/ L (15.3-52.7 nmol/ L), fasting serum cortisol level was 56.3±18.9 nmol/ L (21.6-73.4 nmol/L) at 8 am in 6 patients. Treatment and prognosis: Symptoms were effectively controlled in all children after 2-3 days of intravenous hydrocortisone treatment. After follow-up to June 2022, 11 cases of PNS were cured, 4 cases of PNS were still under treatment, and there was no recurrence of AC. Conclusion Primary nephrotic syndrome in children with adrenal crisis is mainly caused by hormone abatement or infection, and the symptoms of digestive and circulatory system are the main manifestations. Early identification and timely treatment have a good prognosis.