Primary biliary cholangitis (PBC), a chronic, autoimmune, cholestatic disease, typically occurs in elderly women and commonly presents with pruritus, fatigue, and cholestasis and its complications. Gastric antral vascular ectasia (GAVE), an uncommon cause of upper gastrointestinal bleeding, leading to transfusion-dependent chronic iron deficiency anemia, as the first presentation of PBC is unusual. We present the case of an elderly female with recurrent melena and transfusion-dependent anemia for a year without any history of jaundice, ascites, or hepatic encephalopathy. Investigations revealed iron-deficiency anemia, elevated transaminases, alkaline phosphatase (ALP), coarse liver, splenomegaly, and portal vein dilatation on ultrasound. An endoscopic evaluation revealed erythematous linear stripes in the antrum suggestive of GAVE, without esophageal or gastric varices. FibroScan (Echosens, Paris, France) revealed advanced F3 fibrosis. Further etiological workup showed positive antinuclear and antimitochondrial antibodies, elevated IgM levels, and negative viral markers (hepatitis B, C, A, and E). Clinically significant portal hypertension was revealed by the hepatic venous pressure gradient (HVPG), while transjugular liver biopsy (TJLB) revealed lymphocytic infiltration of bile duct epithelium with the destruction of small and medium-sized bile ductules. Iron supplementation, low-dose ursodeoxycholic acid, and argon plasma coagulation were used to treat the patient. At the three-month follow-up, no melena was reported and her hemoglobin and liver function tests remained normal. Patients with PBC presenting with GAVE and recurrent melena as a presenting symptom are rarely reported. An awareness of this presentation is important for its early diagnosis and effective treatment.