Osteoblastoma-like osteosarcoma (OBLOS), is a rare malignant lesion with risks of both local recurrence and distant metastasis. It is considered a rare variant of osteosarcoma. The distinction between (OBLOS), and aggressive osteoblastoma is still debatable and challenging. Based on the initial clinical, radiological, and histological findings, a definitive diagnosis might only sometimes be achievable. On the other hand, the significance of a proper diagnosis must be accomplished because the approach to treatment varies greatly depending on the type of lesion. Sufficient tissue sampling is essential to provide the proper diagnosis. In clinicopathological and radiological discordance cases, a high index of suspicion and significant experience are requirements for an appropriate diagnosis. The OBLOS is categorized as a conventional (high-grade) osteosarcoma in the World Health Organization (WHO) classification system. However, several cases that have been published have been identified as low-grade malignant tumors. There are no precise morphological criteria to discriminate between low- and high-grade lesions. We describe a case of osteoblastoma-like osteosarcoma in an 11-year-old boy involving the right proximal humerus. We provide a brief review of the pertinent literature including diagnosis, differential diagnosis, debatable grading of this type of tumors, management, and prognosis.