The aim of the study was to describe a case of Lisch epithelial corneal dystrophy (LECD), review its clinical and histopathological features and diagnostic imaging, and introduce a novel treatment approach using topical 5-fluorouracil (5-FU).Methods: A 65-year-old woman presented with a recurrent leftsided corneal lesion consistent with LECD. The lesion was evaluated clinically, with high-resolution optical coherence tomography (HR-OCT), and histologically. The lesion was successfully treated with two 1-week cycles of topical 5-FU.Results: Slit-lamp examination showed an opalescent, whorlshaped corneal lesion. HR-OCT revealed a trapezoidal area of normal thickness epithelial hyperreflectivity. Histopathology demonstrated a mucosal epithelium with foamy cytoplasm and increased cell size consistent with LECD. Treatment with topical 5-FU resulted in marked clearance of the corneal lesion on slit-lamp examination and HR-OCT.Conclusions: 5-FU may be considered as a treatment option for LECD.