Background <p>Optic disc melanocytoma is a benign, pigmented tumour of the optic nerve head that is generally stable and asymptomatic. However, in rare cases, these tumours may be complicated by vascular changes and optic disc oedema, mimicking malignant optic disc lesions, resulting in unnecessary invasive interventions. Herein, we present a case of such a tumour presenting with disc oedema and juxtapapillary haemorrhage.</p> Case presentation <p>A 36-year-old woman presented with a 2-week history of blurred vision and floaters in the right eye. Fundus examination revealed a densely-pigmented optic disc lesion associated with optic disc oedema and juxtapapillary haemorrhage. Fluorescein angiography revealed early hypofluorescence, with late staining of the lesion and mild peripapillary vascular abnormalities. Optic disc melanocytoma was diagnosed based on the characteristic clinical and angiographic findings. Considering the benign appearance of the lesion, as well as the potential risks of optic nerve biopsy, conservative management with close observation was the chosen method. During the 6-month follow-up, the lesion remained stable, while visual acuity improved, and optic disc oedema and haemorrhage resolved completely.</p> Conclusions <p>In rare cases, optic disc melanocytoma may present with optic disc oedema and juxtapapillary haemorrhage, raising concerns about malignant transformation. Careful multimodal assessment and short-term follow-up can help to confirm the benign nature and avoid unnecessary invasive procedures.</p>