We report a case study of 2 male pediatric patients presenting with anterior uveitis and elevated renal function parameters. Both were diagnosed with tubulointerstitial nephritis and uveitis (TINU) syndrome and subsequently developed diffuse cerebral symptoms like headache, fatigue and dizziness. Magnetic resonance images (MRI) of the brain showed T2-hyperintense lesions with and without gadolinium enhancement leading to brain biopsy and diagnosis of CNS small vessel vasculitis in both cases. Both patients were treated according to BrainWorks CNS small vessel vasculitis protocol and symptoms vanished over the course of treatment. Follow-up MRIs up to 12 months after initiation of therapy showed no signs of recurrence indicating a monophasic disease.