BackgroundTubular ectasia of rete testis (TERT) is a benign condition due to dilatation of the rete testis as a consequence of multiple etiologies, including postinfectious, trauma, prior inguinal or scrotal surgeries, mechanical compression of extratesticular excretory ducts, and congenital malformation. TERT is a rare and underreported condition in the medical literature. Its association with infertility is lacking in the literature.Case PresentationWe present a rare case of primary infertility and obstructive azoospermia associated with TERT. We performed extensive literature regarding the possible etiologies, associations, clinical diagnosis, and different management options of this entity. Our patient is a 33‐year‐old man presented with primary infertility. He was found to have obstructive azoospermia. During scrotal exploration, the diagnosis of intratesticular obstruction due to TERT was made. The couple was advised to undergo intracytoplasmic sperm injection using testicular sperms. This is the third report of TERT associated with azoospermia.ConclusionTERT is usually asymptomatic but can occasionally be associated with pain or abnormal semen analysis, but further studies are required to confirm its effect on fertility. Fortunately, it has a benign course and typically does not require surgical intervention. It is vital to differentiate it from neoplastic conditions.