Chondroblastic osteosarcoma, representing about 25% of osteosarcoma, is a fatal primary malignancy of the skeleton if not diagnosed and treated appropriately. It most commonly occurs in the long bones of the extremities near the metaphyseal growth plates. In this report, we describe the occurrence of chondroblastic osteosarcoma involving the left distal tibia in a 14-year-old male. The diagnosis was confirmed by the histological examination of a surgical biopsy. The patient was treated by both surgery and neoadjuvant chemotherapy. No recurrence was noted at 3 years of follow-up. To our knowledge, only two cases describing chondroblastic osteosarcoma of the distal tibia had been reported through English medical literature. Therefore, the aim of our article is to make the clinician aware of this rare clinical presentation and also to provide a comprehensive review of the literature related to this uncommon malignant tumour.