The incidence of atypical manifestations of pediatric APSGN has been on the rise in recent years. According to the literature, atypical presentations include subclinical, extrarenal symptomatic, massive proteinuria, and acute nephritis types. In the case of subclinical acute glomerulonephritis, the clinical symptoms are usually mild or absent, and only abnormal urine routine, abnormal ASO, and dynamic changes in C3 are present, so the diagnosis is easily missed. Extra-renal symptomatic nephritis usually has other systemic symptoms outside the kidney as the first or prominent manifestation, while the urinalysis is normal or the changes are mild, so it is easy to be misdiagnosed clinically. For example, APSGN patients with severe edema may develop unilateral or bilateral pulmonary edema, and those with dyspnea and respiratory distress as the first symptoms may be misdiagnosed as pneumonia or heart failure, etc. APSGN patients with sudden rise in blood pressure may develop hypertensive encephalopathy, and may have convulsions, impaired consciousness and blackness as the first manifestations, which may be misdiagnosed as epilepsy or encephalitis, etc. APSGN may also involve the central nervous system and lead to encephalopathy, which may manifest as nausea, vomiting, cognitive changes, etc. The manifestations include nausea, vomiting, cognitive impairment, seizures and visual disturbances, which are considered to be related to hypertension, uremic toxins and cerebral vasculitis. APSGN has been reported to cause reversible posterior encephalopathy syndrome, which is a clinical syndrome with headache, seizures, visual disturbances, consciousness and mental disturbances as the main clinical symptoms and reversible posterior white matter damage as the main imaging manifestation. Therefore, children with rapid clinical appearance of convulsions, coma and heart failure should be considered as possible cases of this disease, and blood pressure should be monitored and urine routine, ASO and C3 should be checked promptly for comprehensive analysis to make early diagnosis and treatment. Massive proteinuric glomerulonephritis may present with moderate to severe edema, nephrotic level proteinuria, and some may have mild plasma albumin decline and hyperlipidemia, which can be easily misdiagnosed as nephritic nephropathy. Recognizing this, the rush to high-dose hormone therapy can be avoided, thus reducing unnecessary suffering of patients. The acute nephritis type progresses more rapidly and can develop severe oliguria, anuria or even acute renal failure within 1 week or 1-2 d. It can be misdiagnosed as acute nephritis, but most of them can be remitted in a short period of time through active symptomatic treatment.