OBJECTIVES:  To estimate the cost-effectiveness of factor VIII+von Willebrand factor combination in treatment of von Willebrand disease in Colombia from the third payer perspective. METHODS: a Markov model was designed which simulates the natural history of the von Willebrand disease for treatment of mild and severe events. The time horizons used were one, three and five years. The model was programed in Excel, complementing it with visual basic. A court of patients with the disease was simulated using monthly cycles. As stages of the model different types of events that a patient with the disease analyzed can present were used. Due to there is no a comparator technology in the health system, the analysis includes three technologies (Wilate®, Immunate® and Haemate P®) using data from weighted averages were. RESULTS: the cost-effectiveness ratio was about 6.45 billion Colombian pesos for the treatment of mild and moderate von Willebrand disease. Individually speaking, treating a patient with this disease would represent costs for mild and moderate patient of $6.2, $17.8 and $28.8 millions, for the first, third and fifth year. Moreover, the ratio of cost-effectiveness of using this combination in seriously ill patients would be $392 millions. This assessment did not establish a direct comparator because in Colombia there are not plasma derivative included in the mandatory health plan for treatment of von Willebrand disease. The effectiveness data and costs were estimated as weighted average from treatment options not included in the mandatory health plan. CONCLUSIONS:  This economic evaluation showed that for winning a year of life in mild and moderate patients would cost $6.4 millions, for the time horizons studied. Moreover for winning a year of life in severely ill patients would represent a significant economic burden for the Colombian health system, however, this technology should be taken into account as a treatment option.