Nora's lesion is a rare benign osteocartilaginous lesion characterized by a mixture of immature bone cells, atypical and bizarre cartilage cells and bland spindle cells. Its pathogenesis remains controversial and its associated signs and symptoms are variable. It is important to identify its clinical, radiological and histopathological characteristics to make the differential diagnosis with other benign or malignant diseases. There are important considerations regarding its diagnosis, which will guide treatment and follow-up. The primary treatment for Nora's lesion is surgical excision. Despite its high rate of local recurrence, the occurrence of metastasis was not reported. We present the case of an adolescent with the diagnosis of Nora's lesion located on the terminal phalanx of the first toe. Our aim is to alert for the existence of this condition in younger ages and in atypical locations and for the need to establish the differential diagnosis with other pathologies, providing the patient the most appropriate treatment.