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Introduction Angiomyolipomas represent neoplasms of mesenchymal origin, made up of abnormal thick-walled blood vessels, smooth spindle muscle cells, and mature adipose cells. The most common site of origin are kidneys, and other localizations are extremely rare. We represent a case of a spermatic cord angiomyolipoma misdiagnosed as incarcerated inguinoscrotal hernia, and to our prudence this is second described case of an angiomyolipoma localized in the spermatic cord. Case outline We present a case of a 63-year-old man presented with high fever and difficulty in walking due to pain and swelling in the right groin. According to the clinical examination and laboratory tests, presumptive diagnosis was incarcerated inguinoscrotal hernia, so the patient was immediately operated on. The exploration of the inguinal canal showed a timorous mass, 9 × 9 cm in size, with the origin from the spermatic cord, so radical inguinal orchiectomy was performed with the removal of the tumor mass. Histopathological and immunohistochemistry examination suggested angiomyolipoma of the spermatic cord. The postoperative course was uneventful. Conclusion Although rare, an angiomyolipoma of the spermatic cord must be included in the differential diagnosis of scrotal masses. Also, we advocate additional diagnostic procedures (ultrasound or computed tomography) for every inguinoscrotal mass before undertaking surgery, since a variety of different causes can be found. After definitive angiomyolipoma diagnosis is obtained, further investigation is needed, especially brain computed tomography due to possible tuberous sclerosis coexistence.