Purpose To characterise malignant conjunctival melanomas with extension and recurrence in the nasolacrimal system.

Methods Localisation of the primary tumour and recurrences of 210 conjunctival melanomas treated in The Netherlands were reviewed for orbital and nasal tumours (1978–2008). Based of these cases and literature data, characteristics for nasolacrimal system extension and metastasis were reviewed.

Results Six patients (3%) showed a recurrence of the primary conjunctival melanoma in the nasolacrimal system. Two of the six primary tumours were limbal tumours; the other four were diffuse tumours involving the fornix. In all six patients, the primary conjunctival melanomas were associated with primary acquired melanosis. During the follow-up period (11.6±3 years, range 3.4-28.5 years, median 8.7 years) two patients developed metastases and died.

Conclusions Patients should be advised to contact their treating ophthalmologist in the case of symptoms of epiphora, nose obstructions and epistaxis, especially non-bulbar and diffuse cases associated with primary acquired melanosis.