Congenital fibrosis of the extraocular muscles (CFEOM) is a genetic disorder that belongs to the congenital disorders of cranial disinervation and is characterized by a non-progressive restrictive ophthalmoplegia. The objective was to present the case of a patient with a CFEOM type 1 diagnosis based on clinical history, physical examination and substantiated by magnetic resonance imaging findings, presenting possible treatments and limitations.