Isolated fallopian tube anomalies are a rare group of disorders, usually observed in otherwise asymptomatic patients. If symptomatic, the patients may present with infertility or unspecified abdominal pains. Various aetiologies have been proposed to explain this phenomenon, ranging from congenital to vascular events. Diagnosis is very challenging because in most cases those abnormalities are not easily seen on ultrasound, computed tomography, or even magnetic resonance imaging. The most objective and certain diagnosis, however, can be obtained through laparoscopy with an associated histopathological examination performed if necessary. Management of this rare group of disorders remains unclear – because the number of cases is relatively small, there is no consensus on treatment. We present a case of a 22-year-old woman with no relevant clinical history and no sexual activity with large bilateral hydrosalpinx that developed as a result of bilateral distal fallopian tube segmental absence with associated occlusion and concomitant endometriotic implants in the vicinity of the left ovarian fossa and the peritoneum of the rectouterine pouch. To our best knowledge, only a few similar cases have been described in the literature. We summarise the available descriptions of this group of pathologies, present theories that have been proposed to explain this phenomenon, and provide various classifications of those disorders.