Cancer and its related therapies are acquired thrombophilic risk factors. Patients with a malignancy can suffer a thrombotic complication such as deep venous thrombosis (DVT) in the lower extremities with a possible following pulmonary embolism. Recently, an increased association between upper extremity DVT and malignancy has been observed and several risk factors were identified. However, few data are available for the association between upper extremity DVT and inherited thrombophilia in oncological patients. Here we report a case of upper extremity DVT complicated by fatal pulmonary embolism in an oncological patient receiving chemotherapy and carrying 2 heterozygous genetic thrombophilic risk factors.