Paraneoplastic acral vascular syndrome (PAVS) is a rare acral vascular syndrome associated with rapid and sudden acrocyanosis or acronecrosis mimicking Raynaud phenomenon. Digital gangrene may occur. PAVS may be a presenting sign of malignancy and has been reported in the setting of adenocarcinoma of the breast, lungs, ovaries, pancreas, and the gastrointestinal system; melanoma; and Hodgkin lymphoma. Metastatic disease is common at the time of diagnosis. The pathogenesis may be multifactorial. Possible mechanisms may include hypercoagulability, tumor embolization, and immune complex-mediated reaction due to carcinogenesis leading to thrombosis, vaso-occlusion, and digital ischemia. PAVS is rare, with an incidence of 2 cases per 100 000 patients annually. The male-to-female ratio is 0.89, with a mean age at onset of 55 years. Most cases resolve following treatment of the underlying malignancy.