BACKGROUNDKimura disease (KD) is a rare, inflammatory, idiopathic condition characterized by painless, slow-growing subcutaneous masses frequently in the head and neck region, 1,2 that occurs more commonly in young males with a peak incidence in their second to fourth decades of life. 3 Renal involvement is the leading systemic manifestation of KD. 2 Different types of kidney disorders have been reported to be related to KD, such as membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerular sclerosis (FSGS), and acute tubular injury. 4 However, the underlying mechanisms of KD and its renalassociated involvement remain unknown. 5 KD diagnosis is often challenging and easily misdiagnosed due to its rarity. 6 So far, different therapeutic approaches, such as surgical excision, immunosuppressive agents (such as thalidomide and cyclosporine), and local radiotherapy, are recommended for KD management. 7,8 However, there is still no specific protocol for definitive KD treatment, and KD patients experience a high rate of recurrence, up to 62%. 9 Here, we reported a 13-year-old male patient who was hospitalized as a result of the nephrotic syndrome relapse and was eventually diagnosed as a case of KD.