An association between glomus tumor and neurofibromatosis type 1 has been reported. It is characterized by multiple tumors and young age at onset. The early diagnosis of neurofibromatosis type 1 is important because it is associated with a high rate of malignancy. A 25-year-old man presented to our hospital with left index finger pain that had persisted for 6 years. Physical and imaging findings suggested a benign soft-tissue tumor. Surgery was performed, and the tumor was pathologically diagnosed as glomus tumor. In this case, the patient with a young-onset glomus tumor was suspected of having neurofibromatosis type 1. An additional medical examination led to the diagnosis of unrecognized neurofibromatosis type 1. We experienced a case in which the onset of a glomus tumor led to the diagnosis of neurofibromatosis type 1. Comorbid neurofibromatosis type 1 should be kept in mind when glomus tumors are diagnosed.