Background: Recent research has identified the coexistence of normal pressure hydrocephalus (NPH) with neurodegenerative disorders, such as progressive supranuclear palsy (PSP). We present a patient with shunt-responsive NPH that was diagnosed with PSP at autopsy, in the absence of the typical clinical features of PSP antemortem. Methods: Medical records were reviewed, including diagnostic imaging and neuropathology. A literature review was conducted. Results: A 78-year-old female presented with a 4-year history of progressive gait dysfunction and cognitive impairment. MRI imaging was consistent with NPH. The patient’s clinical status improved significantly following high-volume lumbar puncture, with TUG test results decreasing to 25.19 sec from 58.73 sec. A lumboperitoneal shunt was inserted. Subsequent clinical improvement was impermanent, despite the absence of shunt malfunction. Autopsy revealed neuropathological findings consistent with PSP. We have identified 7 similar cases of NPH patients found to have concomitant PSP at autopsy in the literature. Like the case we present, these shunted patients experienced an initial, but unsustained, improvement in clinical status. Conclusions: We recommend the clinical management of NPH patients includesurveillance for neurodegenerative disorders. CSF diversion in NPH patients who develop concomitant PSP benefit from CSF diversion, offering them an interval of significantly improved quality of life.