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Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity. We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic ischemic encephalopathy after influenza A Infection with septic shock and severe acute respiratory distress syndrome, followed by meningitis at the age of 10 months. In consequence she developed a severe cerebral atrophy and postmeningitic hydrocephalus requiring placement of a VP shunt. At age 4 she was admitted with community acquired mycoplasma pneumonia and developed increasing pleural effusions leading to severe respiratory distress and requiring continuous chest tube drainage (up to 1000 -1400 ml/day), that could not be weaned. ß trace protein, in CSF present at concentrations > 6 mg/l, was found in the pleural fluid at low concentrations of 2.7 mg/l. An abdomino-thoracic CSF fistula was finally proven by single photon emission computerized tomography combined with low dose computer tomography. After shunt externalization, the pleural effusion stopped and the chest tube was removed. CSF production rate remain high above 500 ml/24h. An atrial CSF shunt could not be placed, since a hemodynamically relevant atrial septum defect with frail circulatory balance would not have tolerated the large CSF volumes. Therefore, she underwent a total bilateral endoscopic choroid plexus laser coagulation (CPC) within the lateral ventricles via bi-occipital burr holes. Postoperatively CSF production rate went close to 0 ml and after external ventricular drain removal no signs and symptoms of hydrocephalus developed during a follow-up of now 2.5 years. In summary, pleural effusions in patients with VP shunt can rarely be caused by an abdomino-thoracic fistula, with non-elevated ß trace protein in the pleural fluid. The majority of reported cases in literature were treated by ventriculo-atrial shunt. This is the 2nd reported case, which has been successfully treated by radical CPC alone including the temporal horn choroid plexus, making the child shunt independent.