Hypertrophic osteopathy (HO) is a rare condition in the horse characterised by symmetrical proliferation of connective tissue and subperiosteal bone, along the diaphyses of limb bones. In this case, a 10-year-old Irish Draught cross Thoroughbred mare was presented for investigation of weight loss, lethargy and firm swelling of all 4 distal limbs. Radiographs of the distal limbs revealed palisade-like periosteal new bone formation perpendicular to the cortex of the distal third metacarpal and metatarsal bones, proximal (P1) and mid (P2) phalanges. Rectal examination revealed a mass of approximately 20 x 30 cm in the right caudoventral abdomen. The mare was diagnosed with HO due to a granulosa thecal cell (GTC) tumour. The GTC tumour was removed and the diagnosis confirmed on histological examination. Clinical examination and radiographs of the distal limbs after surgery demonstrated reduction in size of the limb swellings and new bone formation. To the authors' knowledge this is the first report of HO due to a GTC tumour in a mare.