Endometrial adenocarcinoma presenting with deep venous thrombosis is an exceptional event more typical of extra-mullerian primary tumors. Metastases to the spleen are also unusual in this setting. In addition, primary endometrial adenocarcinoma with signet-ring cell features has been reported only once. This study describes a case of primary endometrial carcinoma with prominent signet-ring cell features that presented in an unusual manner: with bilateral deep vein thromboses and splenic metastases. It is important for clinicians and pathologists to be aware of this entity; it may have an atypical clinical presentation and the histologic features raise a spectrum of differential diagnoses.