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OBJECTIVE To retrospectively evaluate the prevalence and clinical progression of wobbly hedgehog syndrome (WHS) and concurrent incidence of neoplasia in a cohort of African pygmy hedgehogs (Atelerix albiventris). ANIMALS 49 hedgehogs. CLINICAL PRESENTATION AND PROCEDURES Medical records of hedgehogs from 7 institutions across the US over a 20-year period (2000 to 2020) were retrospectively reviewed. Inclusion criteria were hedgehogs of any sex or age with postmortem CNS histopathology consistent with WHS. Collected data included sex, age at onset and euthanasia, major histopathologic findings, reported neurologic clinical signs, and treatments administered. RESULTS 24 males and 25 females were included. Fifteen of 49 (31%) individuals had subclinical WHS with no reported antemortem neurologic clinical signs. In neurologically affected (clinical) hedgehogs (n = 34), the mean ± SD age at onset was 3.3 ± 1.5 years with a median (range) time from onset to euthanasia of 51 days (1 to 319 days). In neurologically affected hedgehogs, the most commonly reported clinical signs were ataxia (n = 21) and pelvic limb paresis (16) and the most commonly administered treatment was meloxicam (13). Overall, 31 of 49 (63%) hedgehogs had a concurrent histopathologic diagnosis of neoplasia outside of the CNS. CLINICAL RELEVANCE The prognosis for hedgehogs with WHS is poor. No treatment had a significant effect on survival time, and neoplasia was a common comorbidity in the current cohort. A small but clinically relevant subset of neurologically normal hedgehogs had a histopathologic diagnosis of WHS.