An intratesticular varicocele is an uncommon entity, and its clinical importance remains unclear. The diagnosis can be made in the case of an intratesticular tortuous vein that shows retrograde flow on color Doppler sonography. The anatomic location and course of the intratesticular varicocele within the testicular parenchyma and its association with the existence of an ipsilateral extratesticular varicocele may vary among patients. The pathophysiologic mechanisms remain unclear; however, it may be questioned whether there is an association between testicular atrophy and intratesticular varicocele development. Although the diagnosis is usually made incidentally, patients may rarely present with acute scrotal pain due to acute thrombosis of an intratesticular varicocele.