Introduction: Iridocyclitis associated with retinitis pigmentosa is rarely described in the medical literature. We report an unusual case of this association.
Observation: A 55-year-old woman with a history of Behçet's disease presented with acute red and painful eyes and moderately blurred vision. Ophthalmologic examination concluded in bilateral acute hypertensive uveitis. Funduscopy and fundus fluorescein angiogram showed retinitis pigmentosa. The same fundus changes were found in the patient's siblings. Recurrences were frequent and topical steroids were always successful.
Conclusion: This case is particular in its association of anterior uveitis as the exclusive manifestation of ocular Behçet's disease and familial retinitis pigmentosa.