Background. Kartagener’s syndrome, a subgroup of primary ciliary dyskinesia, is characterized by situs inversus totalis, chronic sinusitis and bronchiectasis. To date, the association of malignant diseases and Kartagener’s syndrome has been reported and all cases except angioimmunoblastic T cell lymphoma in a child have been seen in adulthood. Case. A 10-year-old boy who was followed with the diagnosis of Katagener’s syndrome, presented with a progressive mass in the cervical region for 6 months. Physical examination revealed mental retardation, multiple lymphadenopathies, the largest in the left cervical region (4x4 cm), and pectus carinatum. Also, on cardiovascular examination, apex beat was felt on the right fifth intercostal space along midclavicular line. Magnetic resonance imaging of nasopharynx showed narrowing of the nasopharyngeal airway with an increase in wall thickness up to 2.5 cm on the posterior wall of the nasopharynx. Also, bilateral multiple cervical lymphadenopathies were noted. The pathological examination of the biopsy from cervical lymphadenopathy revealed a diagnosis of undifferentiated nasopharyngeal carcinoma. Chemotherapy was started for nasopharyngeal carcinoma chemotherapy regimen including cisplatin, docetaxel, and 5-fluorouracil. After four cycles of chemotherapy there was a significant regression in nasopharyngeal mass and lymphadenopathies. The patient underwent radiotherapy to the nasopharynx and bilaterally cervical regions. The patient has been in follow-up for 6 years well and tumor free. However, he is still under the supervision of the pediatric immunology and allergy departments due to recurrent respiratory infections and sinusitis. Conclusion. We present a case of nasopharyngeal carcinoma which developed in a child with Kartagener’s syndrome. To our knowledge, this is the first report of nasopharyngeal carcinoma in a child with Kartagener’s Syndrome.