Primary vaginal leiomyosarcoma in pregnancy is an extremely rare disease which continues to have a poor prognosis. This is due to the late diagnosis as well as the treatment is based on limited experience based on case reports and not randomised trials. The authors report the first case of leiomyosarcoma of the vagina in a pregnancy in a 31-year-old Afro-Caribbean multigravida at the Mt. Hope Maternity Hospital. Despite the administration of systemic chemotherapy and irradiation, the patient succumbed to her illness 11 months after the initial diagnosis. If the prognosis is to be improved, it is prudent that healthcare providers must be more aware and knowledgeable of this tumour and be proactive in its management.
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