Introduction: Enchondroma is a solitary, benign, and intramedullary cartilaginous tumor occurring most commonly in small bones of hands and feet contributing to 3–10% of all bone tumors. They originate from the growth plate cartilage which later on proliferates to form enchondroma. Lesions are central or eccentric and metaphyseal involvement is most common for long bones. We report a case of atypical occurrence of enchondroma in the head of femur in a young male. Case Report: A 20-year-old male patient presented with a history of pain in the left groin for 5 months. Radiological examination showed a lytic lesion in the head of femur. The patient was managed by safe surgical dislocation of the hip, curettage with autogenous iliac crest bone graft with countersunk screw fixation. Histopathology confirmed the lesion to be enchondroma. At the latest follow-up after 6 months, the patient was symptom-free and there was no evidence of any recurrence. Conclusion: Lytic lesions in the neck femur can have a good prognosis provided timely diagnosis and interventions are done. The present case of enchondroma in the head of femur represents a very rare differential diagnosis for the same and this must be kept in mind. So far, no such case has been reported in the literature. Magnetic resonance imaging and histopathology are of the essence to confirm this entity. Keywords: Enchondroma, atypical presentation, safe surgical dislocation of hip.
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