This is a case report of a 31-year-old lady who is known to have ulcerative colitis for 15 years and was on mesalazine. She presented with periorbital swelling, sinusitis, epistaxis, and was found to have positive anti-neutrophil cytoplasmic antibody and anti-proteinase-3 of a high titer. Biopsy from the maxillary sinus showed chronic non-specific inflammation and biopsy from the periorbital fat revealed inflammation and granulomatous changes. She had no other organ involvement. She was diagnosed with a limited form of granumatosis and polyangiitis and treated with methotrexate and prednisolone. The symptoms disappeared after treatment.