Tumours with myogenic, histiocytic and fibro-histiocytic differentiation rarely involve the uveal tract. They may resemble malignant lesions such as melanoma and pose considerable challenges in diagnosis if a biopsy is not performed. Historically, many such lesions were diagnosed post-enucleation, but a high index of suspicion and appropriate imaging and intraocular or nonocular biopsy may allow eye-retaining treatments to be offered in such cases.