We report a 63-year-old woman with common variable immunodeficiency (CVID) and an indolent distal sensory neuropathy. Despite having negative serologic tests for Sjögren's syndrome, evaluation of the neuropathy led to a new diagnosis of Sjögren's syndrome based on the presence of sicca symptoms, an abnormal Schirmer's test, and histologic evidence of sialadenitis. In addition, a thymoma was discovered. We conclude that the occurrence of thymoma, CVID, and Sjogren's syndrome reflect a state of systemic autoimmune dysregulation in this patient. We also reiterate the diagnostic importance of salivary gland biopsy in patients with sicca symptoms and sensory neuropathy.