Full text article. Also available at https://dx.doi.org/10.4314/rjmhs.v3i1.3Background: Paediatric head and neck cancers represent an important group of childhood cancers that require maximum attention at large. They are often diagnosed at advanced clinical stages at Muhimbili National Hospital (MNH). Objective: To describe the clinical profile of paediatric head and neck cancers at a tertiary hospital in Tanzania Method: A hospital based descriptive cross-sectional study was conducted involving 180 paediatric patients. Data were analyzed using SPSS program version 21. Results: A total of 180 paediatric patients were recruited where 61.1% were males and 38.9% were females. Majority belonged to the age group 0-5 years (53.9%). A total of 10 primary anatomical sites were found with the neck (cervical lymph node) predominating (36.1%), followed by the orbit/eye (34.4%) and the least anatomical sites were oral cavity (1.1%) and parapharyngeal space (0.5%). Leukocoria (93.9%), red eye (93.9%) and orbital cellulitis (77.6%) predominated in retinoblastoma but in patients with lymphomas, predominant features were night sweats (100%), weight loss (100%) and fevers (95.8%). Conclusion: The clinical profile depicted in this study appears to correlate with advanced clinical stages.