Neuroendocrine carcinomas of the uterine cervix are rare tumors with aggressive behavior. They comprise <4% of cervical carcinomas. They may coexist with both adenocarcinoma and squamous cell carcinoma of cervix. Signet ring carcinoma of cervix is a rarer entity and less than 20 cases have been described in the literature. We present a case of a 34-year-old female who presented with systemic thrombosis, splenic mass and a cervical mass which on biopsy showed divergent differentiation of primitive large cell neuroendocrine carcinoma with signet ring cells. The cervical tumor was positive for human papilloma virus 16/18 by in situ hybridization, confirming cervical origin of the tumor. This unusual presentation and morphology needs to be recognized and appropriately evaluated when patients present with tumors of unknown origin in metastatic sites.