Background: Sarcoidosis is a systemic infl ammatory disease characterized by non-caseating epitheloid granulomas; whereas it usually involves the lungs and lymph nodes, genitourinary involvement is extremely rare. For this reason, no consensus regarding the diagnostic and management options for testicular sarcoidosis has been reached. Objective: The aim of this case report was to describe an unusual clinical presentation of sarcoidosis. An 8-year-old boy presented with a 6-month history of painless scrotal enlargement and a 2-month history of photophobia, blurred vision, and pain in both eyes. Physical examination revealed tearing, conjunctival hyperemia, and nodular lesions in both eyes as well as bilateral, nontender, irregularly shaped, and very hard testes. Laboratory investigations revealed elevated serum angiotensin-converting enzyme levels, and 8 fl uoro-2-deoxyglucose positron emission tomography (FDG-PET) showed intense FDG accumulation in both testes. Testes biopsy showed non-caseating epitheloid granulomas, staining negative for acid-fast bacilli and fungi; tuberculosis culture was negative. A diagnosis of sarcoidosis was made. Conclusions: This is the fi rst reported case of testicular sarcoidosis in a Turkish child. Frozen section assessment is the most sensitive and reliable method for differentiating testicular masses. The diagnosis of testicular sarcoidosis should be made after excluding other infl ammatory diseases characterized by non-caseating epitheloid granulomas.