Background. Congenital fibrosis of extraocular muscles (CFEOM) is a very rare congenital condition, characterized by variable amounts of restriction of the extraocular muscles, with or without ptosis. The aim of this report was to deseribe a severe, atypical, exposure-induced corneal stromal lysis in two patients. Case report. A mother and a daughter with a severe CFEOM were presented. The surgery of both extraocular muscles and ptosis led to a fair outcome in mother even 30 years after, and a very good outcome in daughter 4 years after the treatment. Conclusion. Though frequently challenging and disappointing the outcome of surgery of both extraocular muscles and ptosis in CFEOM can be favorable even in rather severe cases. To the best of our knowledge, the atypical keratolysis we described has not been highlighted in the literature on CFEOM so far.