Kartagener's syndrome and granulomatous cheilitis are rare entities and are rarely discussed in the literature, and when still associated with the facial manifestation of actinomycosis, triggers severe aggravations in the patient's systemic and oral conditions. The objective of this study is to highlight the importance of multiprofessional work for an early diagnosis, as well as to emphasize the need for knowledge of the patient's systemic and oral health for a safe and quality care. It is also necessary to carry out more detailed studies on the correlation of Kartagener's Syndrome, granulomatous cheilitis and other manifestations, both oral and general, in order to establish effective and recommended therapeutic conducts in the conduction of the treatments, offering the patient integral health promotion and improvements in quality of life. This present case report consists of clinical and laboratorial assistance for diagnoses and treatment behaviors of the infantile patient. The clinical examination evaluated the systemic health, severity of the infection, volumetric increase of the upper lip and constant drainage of the extra-oral fistula with odor (sulfur granules). The patient had numerous foci of infection as residual roots of deciduous teeth, besides the tooth from which the fistula originated, and oral hygiene totally compromised. Laboratory tests were performed for differential diagnosis of leishmaniasis (Montenegro's reaction) for lip lesions - taking into account the origin of the patient from the northern region of the country, an endemic region in Brazil (Piauí), and incisional biopsy of the lip to confirm the diagnosis of granulomatous cheilitis. A prophylactic antibiotic was prescribed for the tooth extraction and drainage of extrabuccal fistula. We found total remission of extrabuccal fistula and significant regression of lip edema after removal of infectious foci. What was noticeable was the emotional improvement of the patient who at the time of diagnosis was generally and psychologically weakened. This is a clinical case that reports the simultaneous presence of three serious and rare factors in a pediatric patient, stressing the essential role of the Dentist, working together with the multidisciplinary team to diagnose, treat and improve the quality of life of the compromised patient
