Background:
Childhood urolithiasis is previously rare but increasingly recognized nowadays. Presentation varies, and often children, do not present with the classical symptoms commonly seen in adults.

Case report:
This case was initially presented to the emergency department with atypical symptoms of nephrolithiasis. Therefore, he was treated for acute appendicitis before an ultrasound abdomen revealed there is gross hydronephrosis, which is secondary to pelvic-ureteric junction obstruction. CT abdomen was then proceeded, found right nephrolithiasis and vesicoureteric calculus with right hydroureter and gross hydronephrosis. Retrograde pyelography (RPG), ureteroscopy (URS), and insertion of the stent for right pelvic-ureteric junction obstruction were performed and subsequently, he developed complications.

Conclusion:
The initial presentation of this case is common however not a classical presentation of renal stone. Therefore, evaluation of the symptoms and initial investigations should be done properly, so that we will not miss this common disease with the rare presentation, especially in children.