PURPOSE: To report the case of a patient with cystoid macular edema (CME) secondary to idiopathic macular telangiectasia (MacTel) type 1, which was successfully treated by cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion. PATIENTS: A 80-year-old man was referred to our department because of a visual defect in his right eye. His best corrected decimal visual acuity (BCVA) was 0.7 (Snellen equivalent [SE], 20/30). METHODS: A fundus examination revealed clustered temporal juxafoveal microaneurysms and foveal CME. The patient refused to undergo conventional treatments, including direct retinal photocoagulation for microaneurysms, intravitreal anti-vascular endothelial growth factor injection, and intravitreal triamcinolone injection. However, he provided consent to undergo cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion. RESULTS: His BCVA was 0.2 (SE, 20/100) just before the surgery. A 27-gauge vitrectomy with internal limiting membrane peeling was performed. Cystotomy was performed during the surgery, and the fibrinogen clot visible in the cystoid cavity was also removed. CME rapidly disappeared after the surgery. Three years postoperatively, the patient had BCVA of 0.5 (SE, 20/40) at the last medical examination, and the CME had not recurred. CONCLUSION: Cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion could be valid treatment options for CME secondary to MacTel type 1.