The authors present a report of a 44-year-old female patient, with complaints of visual disturbances in the left eye. The symptoms were present for at least 5 years and worsened in the last 2 years, impairing her activities. The patient reported perception of a floating circle, which blurred her vision. There was no previous history of trauma, infectious or inflammatory disease. Clinical evaluation included physical examination, laboratory testing, abdominal ultrasonography, thorax X-ray and head tomography. Ophthalmologic examination consisted of visual acuity, motility tests, biomicroscopy, tonometry and indirect ophthalmoscopy. Complementary investigation was done with a and b scan ocular ultrasonography. The chosen therapeutic approach to excise the cyst was pars plana vitrectomy, successfully performed. Anatomopathologic analysis revealed a pigmented vitreous cyst, of possible congenital origin, described as a cystic choristoma from the primitive hyaloid system. Benign evolution, clinical findings and histopathological analysis corroborated the diagnostic hypothesis of a cystic choristoma of the primitive hyaloid system. Surgery (pars plana vitrectomy) was successful and the patient returned with visual acuity of 20/20 in both eyes, and with no further complaints.
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