Objective: We report a case of fibromatous periorchitis, a rare entity that may have differential diagnosis problems with other lesions.

Methods: Our patient underwent a surgical procedure with resection of the tunica vaginalis testis after intraoperative histological evaluation.

Results: Findings observed in both intraoperative biopsy and specimen histopathological study were key for diagnosis.

Conclusions: Although macroscopic appearance of the lesion can be of great value, fibromatous periorchitis diagnosis is based on the histologic features, being important to avoid erroneous therapeutic decisions.

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