The present study investigated the effectiveness of cognitive-behavioral therapy in youth (N = 27) diagnosed with a principal anxiety disorder and school refusal (SR; denial to attend school or difficulty remaining in school). Scant research examines the effectiveness of cognitive-behavioral therapy for treatment-seeking youth with a primary anxiety disorder and comorbid SR. Effects for youth who completed treatment (N = 12) ranged from d = .61 to 2.27 based on youth- and parent-reported anxiety and depressive symptoms, as well as independently rated global functioning. A discussion of treatment drop-out, a case illustration, and treatment recommendations are provided.