A 31 year old man with spina bifida and a ventriculo-atrial (VA) cerebrospinal fluid (CSF) shunt in situ presented with sudden onset dyspnoea and pleuritic chest pain. He reported a nine month history of increasing breathlessness. Clinical signs of right heart strain were present. Echocardiography estimated the pulmonary artery pressure to be 86 mm Hg. Computed tomography pulmonary angiogram confirmed acute pulmonary embolism (PE) and chronic pulmonary thromboembolic disease. He was anticoagulated but unfortunately had a cardiac arrest and succumbed. VA CSF shunts were used for the treatment of hydrocephalus between the 1950s and 1980s. Although most VA shunts sited for hydrocephalus in childhood have been removed, some may remain in situ in adults. These patients are at risk of PE and development of chronic thromboembolic pulmonary hypertension (CTEPH). Removal of the VA shunt should be considered when patients are shown to be shunt independent. We advise the regular screening of patients with VA CSF shunts for pulmonary hypertension with pulse oximetry, electrocardiography, chest radiography and echocardiography as it is preventable, detectable and treatable.