An otolaryngologist is conversant with the potential of middle-ear disease leading to lateral sinus thrombosis and subsequent jugular vein thrombosis, although this is becoming an increasingly rare event. Spontaneous jugular vein thrombosis from non-otological causes, however, is not well described in the otolaryngology literature. We present three cases of jugular vein thrombosis who presented primarily to the Otolaryngology department each attributable to a different pathology including discoid lupus erythematosus, antiphospholipid syndrome and metastatic malignancy. Awareness needs to be increased of this condition in the differential diagnosis of acute neck swelling and neck pain.