Mycoplasma pneumoniae is a common pathogen causing respiratory infections in children and adults. In addition to respiratory diseases, Mycoplasma pneumoniae is also involved in numerous extrapulmonary diseases. Thrombosis is an extrapulmonary manifestation associated with Mycoplasma pneumoniae infection. In recent years, an increasing number of case reports have been published identifying thrombosis secondary to Mycoplasma pneumoniae infection. In the present study, the available relevant literature in English available on PubMed, Medline and Web of Science was consulted. The results of the present study demonstrated that in patients with thrombosis caused by Mycoplasma pneumoniae infection, some of the factors causing thrombosis are transient and some are due to hereditary thrombophilia. Following timely treatment, the majority of patients recovered completely but some patients had a poor prognosis. The present review focuses on the pathogenesis, clinical features, treatment and prognosis of this crucial issue, which contributes toward the understanding of the disease.