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To evaluate the recurrence of Thiel-Behnke dystrophy (linked to the 10 q23-q24 locus) after phototherapeutic keratectomy or penetrating keratoplasty.This is a retrospective study of 4 patients (8 eyes) who underwent phototherapeutic keratectomy and 1 patient (2 eyes) who underwent penetrating keratoplasty. Best corrected visual acuity was assessed, and biomicroscopic examinations for evidence of recurrent dystrophy were documented and photographed. The location, lesion distribution, and lesion pattern of any recurrence was noted.Follow-up ranged from 8 months to 25 years (mean ± SD 9.7 ± 7.97 years). All 10 eyes showed biomicroscopic evidence of central recurrence. Six eyes showed an intermediate zone of honeycomb opacities as well as a peripheral zone of focal and geographic lesions. Despite the high incidence of recurrence, functional central visual acuity was maintained. All eyes maintained functional best corrected visual acuity (ranging from 20/25 to 20/80) despite the postoperative recurrence.Recurrence of Thiel-Behnke corneal dystrophy is extremely high after either phototherapeutic keratectomy or penetrating keratoplasty. Despite the high incidence of recurrence, the central cornea is the last to be affected. The peripheral-to-central progression of the lesions points to an epithelial origin for the pathogenesis of the dystrophy. Phototherapeutic keratectomy in the treatment of Thiel-Behnke corneal dystrophy offers a safe and effective treatment modality, providing patients up to 8 years of improved vision ranging from 8 months to 8 years (mean ± SD 3.7 ± 2.7 years) and delaying or circumventing the need for more invasive intraocular surgical intervention.