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Background: The fluoropyrimidine anticancer drug, especially 5- fluorouracil 5-FU and its prodrug capecitabine are still being the backbone of chemotherapeutic regimens for colorectal cancer. Patients and methods: A year-old lady with resected stage III carcinoma of sigmoid colon was scheduled to have a course of 5-FU based adjuvant chemotherapy. She developed unexpected acute severe grade 4 toxicity after the first cycle of chemotherapy. To the best of our knowledge, this variant is a novel pathogenic splicing variant of the DPYD gene resulting in a non-functional allele. We then titrated the 5-FU dose, and she tolerated the subsequent cycles of chemotherapy and completed the whole course of adjuvant chemotherapy. Conclusions: With a pre-emptive test on DPD deficiency before the administration of the fluoropyrimidine drugs, the aforementioned patient's life-threatening event could be avoided.
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