Wilson disease (WD) is a rare autosomal recessive disorder of copper transport with copper accumulation in various organs. The clinical presentations of WD are variable. The diagnosis of WD can be challenged when patients show clinically rare symptoms. This report described a case of identical twin brothers diagnosed with WD who initially presented with mild-moderate microscopic hematuria and had a consequent delay in the diagnosis and treatment. The 6-year-old younger brother initially presented with hematuria before he was admitted to our hospital at his age of 11. He was found with mild gait disturbance in physical examination upon admission. Further evaluation revealed abnormalities including laboratory investigations of cooper metabolism, brain MRI, liver ultrasound, and Kayser-Fleischer (K-F) rings. The older twin brother presented with hematuria and proteinuria without hepatic and neurological symptoms at age 11. K-F rings were also confirmed. Genetic testing was performed on the twins and revealed mutations of R778L and A874V on both alleles of ATP7B gene. The diagnosis of WD was made on the twins according to the Leipzig scoring system. The twins regularly received sodium dimercaptopropane sulphonate (DMPS) treatment, supplemented with a Chinese traditional medicine, called Gandou decoction. The symptoms of the twin brothers were improved after treatment. The two patients had been reported no renal, hepatic, or neurological abnormalities during a 5.5-year follow-up period. The study demonstrates that WD should be considered as one of differential diagnoses when long-term renal abnormalities with unknown etiology occur in pediatric population. An early diagnosis and treatment are essential for the WD patients to avoid irreversible multi-organ injury.