Angiomyolipoma (AML) is a benign mesenchymal tumor commonly arising in the kidney and rarely encountered in extrarenal sites. Testicular involvement is exceedingly rare, with only a few cases reported in the literature. We report the case of a 43-year-old man presenting with hematuria and dysuria, in whom a left testicular mass was incidentally identified. Imaging suggested a primary testicular neoplasm, and radical orchidectomy was performed. Surgery was uneventful. Histopathological examination revealed a well-circumscribed lesion composed of mature adipose tissue, smooth muscle bundles, and thick-walled blood vessels. Immunohistochemistry demonstrated positivity for smooth muscle markers, while melanocytic markers showed focal weak positivity. No evidence of germ cell neoplasia was identified. These findings supported the diagnosis of testicular angiomyolipoma. Testicular angiomyolipoma is an exceptionally rare entity that may mimic malignant testicular tumors clinically and radiologically. Accurate diagnosis relies on recognition of characteristic morphological and immunohistochemical features, highlighting the importance of clinicopathological correlation.
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