A 6‐year‐old, male, neutered, brown eyed labrador retriever was presented with an intraocular pale pink mass in the left eye. Enucleation of the globe was performed, and the mass was submitted for histopathology. Histology and immunohistochemistry confirmed the mass as a malignant nerve sheath tumour. There was no evidence of mass recurrence, and general and neurological examination remained normal at 9‐month follow‐up. While the majority of intraocular neoplasms are benign, a malignant neoplasm must be considered in all cases of intraocular mass. Differentiation between benign and malignant intraocular neoplasms is challenging and often requires enucleation with histopathology to confirm diagnosis. Tumour staging should be considered in the event of a malignant nerve sheath tumour. Finally, uveal malignant nerve sheath tumour should not only be considered in blue eyed dogs, but also in brown eyed dogs as in the present case.