BackgroundInformation about the clinical and clinicopathologic aspects of cerebrospinal nematodiasis (CN) in camelids is limited.HypothesisClinical and therapeutic variables will be identified as factors predictive of survival.AnimalsClient‐owned camelids suspected of havingCNadmitted to Purdue University between 1995 and 2015.MethodsA retrospective study was performed. A diagnosis ofCNwas based on cerebrospinal fluid (CSF) eosinophilic pleocytosis or postmortem findings.ResultsEleven alpacas and 9 llamas met the inclusion criteria. Seventy‐five percent of the camelids were male (27% castrated and 73% intact). Common clinical abnormalities included proprioceptive deficits (100% of animals), recumbency (55%), tachypnea (55%), and ataxia (40%). Among the 85% of treated animals, 100% receivedPOfenbendazole, and 88% received a nonsteroidal anti‐inflammatory drug. The survival rate to discharge was 45%. Plasma fibrinogen concentration, creatine kinase activity, and serum creatinine concentration were significantly higher in nonsurvivors. Blood eosinophil count, platelet count, and totalCO2were significantly lower in nonsurvivors. Factors associated with survival were species, sex, absence of treatment with corticosteroids, and clinical improvement. There was no association between recumbency at admission and survival. A plasma fibrinogen concentration above >266 mg/dLwas an excellent diagnostic test to predict survival in the presence of neurological signs orCSFeosinophilia.ConclusionsAlthough prognosis forCNin camelids is guarded, presence of recumbency at admission is not predictive of nonsurvival. Male camelids and llamas appear more likely to die fromCN. Corticosteroid treatment is contraindicated in animals diagnosed withCN.