The purpose of this paper was to describe a patient with pericentral retinitis pigmentosa (RP) and cystoid macular edema (CME) refractory to oral acetazolamide alone who was successfully treated with adjunctive pegaptanib sodium. A 33-year-old man presented with decreased vision and a best-corrected visual acuity (BCVA) in his left eye of 20/200 due to CME secondary to RP. Although he had been treated daily for 1 month with 500 mg of oral acetazolamide, the edema was unresponsive. When informed of the available treatment options, the patient requested adjunctive intravitreal pegaptanib sodium. One month after receiving an injection of pegaptanib sodium 0.3 mg and continued daily acetazolamide, the patient's BCVA had improved to 20/40. At the 4-month follow-up visit, no recurrence of CME was found on fundus biomicroscopy, fundus-related perimetry, and optical coherence tomography. We conclude that intravitreal pegaptanib sodium combined with daily doses of acetazolamide appears to provide benefits in CME refractory to oral acetazolamide alone with regards to the improvement of visual acuity.