Non-ossifying fibroma (NOF) is a common benign bone tumor with a high probability of occurrence in children and adolescents.It is commonly seen in the metaphysis of long bones, eccentrically located, and can coexist with other malignant tumors such asneuroendocrine tumors (NET). To date, plain radiographs play a major role in the diagnosis of these benign bone tumors. Herein,we report the case of a 13-year-old male patient who was diagnosed with pulmonary NET and underwent right lung lobectomyfor a hilar mass which later revealed a well-differentiated NET. The follow-up 68Ga DOTA-TOC PET/CT showed a focalsomatostatin receptor expression in the left distal femur, with corresponding CT component findings of a well-defined osteolyticbone lesion located within the medial aspect of the left distal femoral metaphysis, strongly indicative of NOF. To the best of ourknowledge, this is the first reported case of such an occurrence.