A 10-year-old Lipizzaner gelding was presented with intermittent ataxia and hindlimb weakness. Ultrasonographic examination identified a mass cranial to the tuber sacrale. A provisional diagnosis of a soft tissue sarcoma was made based on a biopsy specimen. Owing to the extensive nature of the tumour and the associated poor prognosis, the horse was subjected to euthanasia on humane grounds. A post mortem examination revealed a locally infiltrative soft mass within the left lumbosacral epaxial musculature. Histologically, an infiltrative neoplasm predominantly composed of pleomorphic spindle or stellate-shaped cells was identified. Neoplastic cells exhibited strong S-100 protein and GFAP expression and variable vimentin, NSE, NGFR and myoglobin expression. They were uniformly negative for pan-cytokeratin, melan A, laminin and desmin. The ultrastructural examination revealed pleomorphic cells with long cytoplasmic processes and an absence of melanosomes. Based on these results, a diagnosis of malignant peripheral nerve sheath tumour was made. This report contributes further information to assist in the diagnosis of these poorly defined neoplasms in animals, especially when they occur in uncommon locations.