Background:The incidence of Pneumocystis jirovecii pneumonia (PJP) in patients with underlying neurological conditions is not well established, and the necessity of PJP prophylaxis for immunocompromised patients with neurological disorders is uncertain.Methods:Single-center retrospective analysis of non-HIV PJP patients at a tertiary referral center from 2007-2016 to determine the incidence of PJP in patients with primary neurological disordersResults:The study included 142 non-HIV PJP patients. Twenty patients had primary neurological diagnoses and 122 had non-neurological conditions. Associated neurological diagnoses included brain malignancies (N=16), myasthenia gravis (MG) (N=2), myopathy (N=2), neuromyelitis optica (NMO) (N=1) and central nervous system vasculitis (N=1). Estimated incidences of PJP were 0.7% for NMO and 0.3% for MG patients for the 10-year study period, while 4.6% of NMO and 3.8% of MG patients were placed on PJP prophylaxis. A survey of 24 neurologists who prescribe immunotherapy or chemotherapy confirmed an infrequent occurrence of PJP in their practice. Malignancy or parenchymal organ failure was present in 90% of neurological PJP patients and coexisting infections occurred in 45%.Conclusion:The overall incidence of PJP in patients with non-neoplastic neurological disorders is exceedingly low, raising doubt about the value of routine PJP prophylaxis in neurological patients outside neuro-oncology. PJP infection occurs frequently in patients with malignancy or parenchymal organ failure, indicating that overall health status may serve as a predisposing factor for PJP.