The paraneoplastic variant of granuloma annulare (GA) is a rare cutaneous manifestation of underlying malignancy that is most commonly associated with systemic lymphoma. We report an interesting case of a patient with gastrointestinal stromal tumour (GIST) of the stomach presenting with extensive generalized GA. GIST was diagnosed 2 months after the diagnosis of GA. Resolution of the GA was seen 1 month after surgical excision of GIST. The close correlation of the clinical courses of these two rare diseases suggests that their coexistence was more than a coincidental finding. This case highlights the importance of excluding paraneoplastic GA, especially in cases where the skin manifestations are extensive and resistant to treatment.