Defects in the Canal of Nuck are rare abnormalities of the female genitalia, which are typically detected and repaired in young age. In the present report, a case of a Nuck cyst in a 40-year old female patient is described. Additionally, the current literature concerning cases of women with hydrocele of Nuck canal was systematically reviewed. A total of 16 case reports of 16 patients with Nuck hydrocele (mean age of 35.18 years), have been reported to date. A right inguinal mass was noted in 13 patients (81.3%) whereas in 3 patients a left-sided mass was noted. The surgical approach was open in 13 cases and laparoscopic in 3 cases. Two cases underwent hydrocelectomy and inguinal ring ligation, whereas in 7 cases a simple cystectomy was performed. In 2 cases the round ligament was excised along with the hydrocele. In one of these 2, ligamentum rotundum necrosis and presence of a haemorrhagic cyst of the canal of Nuck were identified. Hernia repair and hydrocelectomy was performed in 5 cases. A cyst of the Canal of Nuck is relatively rare, but should be considered during the diagnosis of inguinal masses in female patients.