Background: Celiac disease (CD) is an immune-mediated, gluten-induced enteropathy with intestinal and extraintestinal manifestations. Chronic urticaria (CU) is a heterogeneous inflammatory skin disorder often considered idiopathic, but emerging evidence suggests possible autoimmune causes. Methods: We describe a pediatric case in which CU and angioedema were the sole clinical expressions of CD. We also conducted a scoping review of the literature to assess the prevalence of CD in CU patients and the therapeutic impact of a gluten-free diet (GFD). Results: The child’s CU resolved rapidly after initiating a GFD, with complete remission and normalization of anti-tissue transglutaminase at follow-up. Literature review shows that CD is significantly more common in CU patients than in the general population, and several case reports document remission of CU after GFD. However, leading guidelines for CD and CU do not currently recommend mutual screening, and pathophysiological mechanisms linking the two conditions remain incompletely understood. Conclusions: Chronic urticaria may be the sole clinical manifestation of CD. Screening for CD in patients with CU may be considered, particularly in those with autoimmune features or disease refractory to standard treatment. Initiating a GFD can lead to rapid symptom remission, reduce dependence on conventional therapies and improve quality of life.