BACKGROUND: Surgical resection of distal cholangiocarcinoma (dCCA) offers the only chance for cure and long-term survival. The current literature provides limited data regarding the surgical management and long-term outcomes of dCCA. This study aims to describe the presentation, management, and outcomes of dCCA at a large academic referral centre in South Africa. METHODS: A retrospective study was performed of all patients who underwent curative-intended surgery for dCCA at Groote Schuur Hospital from 2000 to 2020. RESULTS: Over 21 years, 25 patients underwent pancreaticoduodenectomy (PD) for dCCA. Most patients were male (68%), and the mean age was 56.8 years. Of the patients, 22 (84%) underwent preoperative biliary drainage (PBD). There were 29 recorded complications in 25 patients; postoperative pancreatic fistula (POPF) and surgical site infection (SSI) each occurred in 24% of the cohort. The mean hospital stay was 17.2 days without perioperative mortality. With none lost to follow-up, the 1, 3, 5, 10, and 20-year survival rates were 84%, 24%, 16%, 12%, and 4%, respectively. Only T3 status was associated with significantly lower overall survival (OS). Age, albumin levels, PBD, margin status (R0 vs. R1), and nodal status (N0 vs. N1/N2) did not influence OS. CONCLUSION: This is the first study detailing the management and outcomes of dCCA from sub-Saharan Africa (SSA). Despite the complete resection of dCCA, the prognosis is poor, and the long-term survival rate in our study is equivalent to that reported in the literature. T3 disease is an important prognostic factor and is associated with poor OS. Surprisingly, nodal disease and margin status did not affect OS in the cohort of patients.