Objective: To report an infrequent case of primary signet ring cell carcinoma of the cervix (PSRCC), review the literature and evaluate the clinicopathological characteristics.

Material and methods: A 51-year-old female patient, with 3 years of disease characterised by gynaecologic bleeding and pelvic pain. On examination, cervix replaced by tumour and infiltrated parametria; with cytology and histology of adenocarcinoma with cells in a signet ring pattern. Disease extension studies were negative. Classified as PSRCC stage IIIB, chemotherapy and radiotherapy were indicated, but the patient died a month later. The bibliographic search included publications up to July 2023.

Results: 32 cases with a mean age of 47.6 years were identified. The most frequent symptoms were uterine bleeding and abdominal and pelvic pain. The initial stages were treated surgically, some with adjuvant chemotherapy and/or radiotherapy, with favourable responses; a difference from advanced cases. The average survival was 17.6 months.

Conclusion: PSRCC is rare, with 32 cases reported according to the review. The expression with immunohistochemical and molecular techniques for the human papilloma virus can help confirm the gynaecological primary, but its diagnosis is one of exclusion, since its morphological pattern is related to other primaries, mainly digestive.