Adenocarcinoma of the rete testis is a rare tumor. Histologic diagnosi
s is difficult, and in the past the tumor may have been incorrectly id
entified in a number of cases, leading to misleading information on th
e nature and behavior of this neoplasm. We present the case of a 39-ye
ar-old man with a long history of a small left hydrocele, who was lost
to follow-up and presented again 2 years later with testicular discom
fort. Sonographic findings were consistent with a testicular tumor. Hi
stology confirmed low-grade adenocarcinoma of the rete testis, the fir
st reported tumor of this grade. Previously reported cases of rete tes
tis carcinoma are reviewed.