Insufficient criteria to distinguish enchondroma from low-grade chondrosarcoma occasionally lead to uncertainty concerning the optimal extent of surgery. For either tumor the clinical outcome is mainly compromised by local recurrence. Reviewing our medical records, we retrospectively identified a female patient who was diagnosed as suffering from a large enchondroma of the left humerus in childhood and could be followed up for almost two decades. Following local excision of the tumor at the age of 14 years, clinical follow-up was uneventful for 20 years. A potential primary extensive excision or amputation would have substantially compromised this young woman's quality of life. The complete preservation of function here following a local excision has stimulated a critical discussion of radical surgical treatment.