Purpose: To describe a case of Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma with infiltration of both lacrimal glands. Observations: A 63-year-old male with Waldenström's macroglobulinemia was referred to the ophthalmological clinic due to intermittent bilateral painless swollen eyelids. The patient had slight bilateral chemosis along with swelling of both eyelids. A mechanical ptosis was present on both sides. Funduscopic examination and tonometry were normal. Computed tomography and positron emission tomography showed an enlargement of both lacrimal glands with positive PET signal, and hence a biopsy was performed for histological and cytogenetic examination. Histopathological examination revealed an infiltrate of lymphoplasmacytic cells and small lymphocytes within the lacrimal gland. The tumor cells stained positive for IgM and CD20, CD79, BCL-2, and kappa light chain. A cytogenetic examination revealed a mutation in MYD88 confirming Morbus Waldenström/lymphoplasmacytic lymphoma. Conclusions and importance: Intermittent swollen lacrimal glands are a rather common symptom, and Morbus Waldenström/lymphoplasmacytic lymphoma should be considered as a differential diagnosis. This symptom should be carefully evaluated in Waldenström patients, as it can be a sign of disease progression in case of lacrimal gland involvement.