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ABSTRACT Introduction: Patients with Purkinje cell cytoplasmic autoantibody type 1 (PCA-1-IgG, a.k.a. anti-Yo) classically present with a paraneoplastic syndrome characterized by subacute onset cerebellar ataxia with rapid progression. Recently, a broader spectrum of neurological presentations has been recognized, which include peripheral neuropathy, but detailed case descriptions are lacking. Methods: A patient presented with a painful progressive multifocal sensorimotor polyneuropathy including face pain. Whole body positron emission tomography (PET), exploratory abdominal laparotomy and nerve biopsy were combined with serial neurological examinations and neuro-immunological testing to diagnose and determine treatment. Results: PCA-1 autoimmunity was identified. Although a PET scan failed to detect a tumor, the patient underwent an exploratory laparotomy, given the strong association of PCA-1-IgG autoantibodies with underlying malignancy. The patient was diagnosed with an ovarian lymphoepithelial cancer that was subsequently treated with chemotherapy. Conclusions: PCA-1-IgG autoantibodies can occur in the setting of peripheral neuropathy and predict type of cancer. Multifocal neuropathy with face pain and treatment-resistance is a specific clinical pattern associated with PCA-1-IgG autoantibodies and ovarian cancer. Muscle Nerve 48:827-831, 2013 [ABSTRACT FROM AUTHOR]