INTRODUCTION: Nerve sheath tumours of the kidney are particularly rare and, in the few reported cases, are all situated in the hilar region. CASE PRESENTATION: We describe the case of a tumour presenting towards the lateral border of the ventral aspect of the mid-zone of the kidney. This was a spindle cell lesion in which the cells strongly and diffusely expressed cytokeratins, but were negative for epithelial membrane antigen. The cells also expressed S-100 protein and glial fibrillary acidic protein, confirming the diagnosis of a cellular schwannoma. CONCLUSION: To the best of our knowledge, this is the first case of a cellular schwannoma presenting towards the lateral border of the kidney. The case also highlights the importance of using a panel of antibodies in diagnosing spindle cell neoplasms in the kidney.