BACKGROUND Cervix mucinous adenocarcinomas have been defined by WHO classification into different subtypes: gastric, intestinal and ring signet cell. Ring signet cell subtype represent a diagnostic challenge due to the small number of cases described in the literature. We report hereby the 31st case worldwide, which is all the more exceptional as its atypical clinical presentation with mainly urological clinical signs represented a real diagnostic challenge. METHODS We present the uncommon case of a multiparous, menopausal 68 years-old-woman, who presented in our department for pelvic pain evolving for 4 months associated with dysuria. Gynecological examination showed a bulging mass on the anterior vaginal wall with mucoid urinary discharge. Perineal ultrasound and pelvic MRI showed an anterior vaginal mass arising from the anterior vaginal wall, invading the bladder, urethra and respecting vagina's upper third and the rectum associated with multiple metastatic left iliac lymph nodes. Anatomopathological analysis revealed a moderately differentiated mucinous adenocarcinoma with a signet-ring cell appearance. IHC stain for P16, marker for high-risk HPV, was strongly positive. Due to the advanced stage, the patient was not a candidate for upfront surgery and received definitive chemoradiation with palliative intent. The patient succumbed to her disease after only one month of chemotherapy. CONCLUSIONS Primary signet ring cell carcinoma of the cervix is rare and associated with a poor outcome. Prognosis is related to the clinical stage. Differentiate primary from metastatic signet cell carcinoma is compulsory. IHC is very helpful but not decisive and the diagnosis is often made by exclusion.