A large abdominal mass in a 3-year-old filly was discovered clinically and removed and examined after the horse had been humanely killed. Histologically, three different tissue types were noted, occurring in equal amounts: epithelium largely in the form of well-differentiated tubules, multifocally distributed cell-dense foci of blastema, and myxoid to fibrous stroma. A diagnosis of triphasic nephroblastoma, a rare tumour in the horse, was made on the basis of microscopical findings. The finding of numerous glomeruloid structures confirmed the diagnosis and assisted in distinguishing the tumour from a teratoma. The histogenesis of nephroblastoma is briefly discussed in the context of the histological and immunohistochemical findings of the case.