Herein we describe the first reported case of epididymal inflammatory pseudotumor (IPT) with incidental sperm granuloma in an adolescent. IPTs of epididymal origin are very rare, with a differential diagnosis including benign and malignant processes. Rhabdomyosarcomas and inflammatory myofibroblastic tumors are important diagnostic considerations that display pathologic similarities to IPTs. These lesions were excluded from diagnosis in this case and the important pathologic features allowing for exclusion are detailed within. Lastly, sperm granulomas are extremely rare pathologic findings in pediatric patients and their presence in this situation is likely the result of downstream tumor obstruction.