Rationale: Kawasaki disease affects multiple organ systems. Its typical symptoms include fever, rash, oropharyngeal mucosal erythema, bilateral non-exudative conjunctivitis, cervical lymphadenopathy, extremity changes, and membranous desquamation of the fingers and toes. In severe cases, cardiovascular, respiratory, musculoskeletal, gastrointestinal, neurological, and genitourinary complications may occur. In the early stage, Kawasaki disease is often manifested by uncommon symptoms, such as pyuria, meningitis, shock, and retropharyngeal or parapharyngeal abscess, which may delay diagnosis and treatment. We have reported a case of Kawasaki disease presenting with mumps and reviewed the clinical features of 14 other similar cases, in order to facilitate the early diagnosis and treatment of this unusual presentation of Kawasaki disease., Patient Concerns: A 10-year-old boy presented with persistent fever and parotitis and was diagnosed with suppurative parotitis. After antibiotic therapy, the parotid swelling reduced, but the fever persisted and other typical symptoms of Kawasaki disease appeared, including bilateral conjunctival hyperaemia, cervical lymphadenopathy, oropharyngeal mucosal erythema, membranous desquamation of the fingers, and left coronary artery widening., Diagnoses: The patient was diagnosed with Kawasaki disease 12 days after the onset of fever., Interventions: The patient was administered γ-globulin 1.0 g/kg·d for 2 consecutive days and oral aspirin 5 mg/kg·d., Outcomes: The left coronary artery returned to a width of 3.8 mm after 1 month and of 3.1 mm after 3 months. The dose of aspirin was reduced to 3 mg/kg·d after 2 months and to 1.5 mg/kg·d after 3 months., Lessons: Physicians should be aware that Kawasaki disease may develop after parotitis.