ABSTRACT.Purpose: To determine the frequency of histologically verified lesions of the lacrimal drainage system in Denmark between the years 1910 and 1999. Furthermore, to correlate the clinical diagnosis with the pathology diagnosis. Methods: Retrospective review of all pathology reports from 1910 to 1999 in the files of the Eye Pathology Institute, University of Copenhagen, describing a lesion of the lacrimal drainage system. In addition, a retrospective review of all reports describing a lesion of the lacrimal drainage system from the Danish Pathology Database. All specimens were re-evaluated, except in cases with a primary diagnosis of dacryocystitis. In these cases a sample of 25% was re-evaluated. Results: A total of 643 lesions were collected. Dacryocystitis was the most frequent lesion, constituting 508 cases (79%). The remaining cases were diagnosed as dacryolithiasis (62 cases; 7.9%), tumour (29 cases; 4.5%), trauma (19 cases; 3.0%), congenital malformation (nine cases; 1.4%), canaliculitis (eight cases; 1.2%) and granulomatous inflammation (eight cases; 1.2%). Seventeen tumours were malignant, of which B-cell lymphoma was the most common (six cases). In 0.6% of cases with a clinical diagnosis of dacryocystitis/lithiasis a nonsuspected malignant tumour was diagnosed. Micro-organisms were uncommon in dacryocystitis (9%) but frequent in cases of dacryolithiasis (87%). Conclusion: Dacryocystitis was by far the most frequent lesion of the lacrimal drainage system referred for histopathological evaluation. Dacryolithiasis was often associated with micro-organisms, especially Gram positive rods. Histopathology is necessary to confirm suspected tumours, more than half of which were inflammatory lesions, and to detect tumours that sometimes masquerade as inflammation.