Granulomatous interstitial nephritis is an uncommon finding in a kidney biopsy. The differential diagnosis is broad and includes infections, drug exposure, and sarcoidosis. Sarcoidosis, a systemic disorder of unknown etiology characterized by the presence of noncaseating granulomata in affected organs, is rare in children. We discuss an adolescent boy with the unusual presentation of granulomatous interstitial nephritis and acute deafness. Sarcoidosis should be considered as part of the differential diagnosis for children and adolescents with hearing loss and kidney disease.