PURPOSE: To demonstrate that hairy cell leukemia may involve the eye. METHODS: We examined a patient who had a unique ocular manifestation of hairy cell leukemia with severe panuveitis in one eye and leukemic retinopathy in the fellow eye. RESULTS: Therapy with 2 chloro-deoxy-adenosine, a drug recently introduced for treatment of hairy cell leukemia, led to dramatic improvement of the ocular complications. CONCLUSIONS: Hairy cell leukemia may involve the eye and ocular symptoms may respond to systemic treatment with 2 chloro-deoxy-adenosine.