Fanconi anemia (FA) is a rare genetic disorder, involving all three blood cell lines. It is the most common cause of inherited bone marrow failure (IBMF) characterized by pancytopenia (1). Abnormalities of FA genes (at least 15 identified) are inherited in an autosomal recessive manner, except for FANCB mutations, which are inherited in an X-linked manner (2). While rare, FA is the most common IBMFS, the incidence has been estimated to be 1 in 300,000 live births, and the prevalence of 1 to 9 per million (3). Mortality in this subset of patients is associated with solid tumors and leukemia, being the leading cause of death (1). We describe the diagnosis of an osteochondroma in an 18-year-old male with FA in Colombia.