Abstract
Low-grade fibromyxoid sarcoma (LGFMS) is a rare slow-growing malignant tumour with a deceptively benign histologic appearance. It typically involves young or middle-aged adults. These tumours usually occur in the proximal extremities and trunk. Sporadically they may be found in unusual locations leading to diagnostic dilemma. We report a 5year old boy that presented with painless right parotid swelling of 6 weeks duration. There was a 12-week presentation-to-diagnosis delay, due to the initial misdiagnosis as Burkitt's lymphoma that was entertained because of the region involved, rate of progression and endemicity of Burkitt's in our setting. Compromise of the aerodigestive tract was a major challenge, in addition to early recurrence and metastasis. We advocate for high index of suspicion, prompt tissue diagnosis and multidisciplinary intervention for better outcome.