We report the history and clinical presentation of an 88-year-old female with Fuchs dystrophy who developed an acute anterior necrotizing scleritis in her left eye 23 months after an uncomplicated combined penetrating keratoplasty and phacoemulsification with intraocular lens implantation which progressed to slceral perforation with uveal prolapses. The patient underwent a complete systemic work-up for both autoimmune and infectious causes of scleritis. Surgical specimens of the area of scleral perforation were sent for histology and microbiologic studies. Analysis of surgical specimens revealed the presence of culture-proven Nocardia asteroides as a causative agent for the patient's scleral perforation. Results of her systemic autoimmune work-up were not conclusive. Successful treatment with tectonic scleral reinforcement with donor corneal tissue and preserved pericardium, oral and topical trimethoprim-sulfamethoxazole and topical amikacin salvaged the globe and increased vision. The patient's final best-corrected visual acuity sixteen months after her last operation remains 20/70. Prompt surgical intervention with submission of appropriate specimens for pathological diagnosis and microbiology, along with consultation with rheumatologic and infectious disease specialists, are mandatory to minimize visual loss in cases of suspected infectious necrotizing scleritis.