Objective This study sought to evaluate the clinical features of the incomplete Kawasaki disease (KD) with cervical lymphadenopathy and fever as initial manifestations.

Methods We conducted a retrospective analysis of the clinical records of consecutive KD patients admitted to our hospital. Children presenting with cervical lymphadenopathy and fever as the initial manifestations, with none or only one another clinical feature of KD were defined as incomplete KD (IKD) group.

Results A total of 1420 patients were included. Thirty-one (2.2%) were classified as IKD. There were no differences in age and gender ratio between two groups. The duration of fever in IKD group (10.3 ± 5.7 d) was longer than that in KD group (6.9 ± 3.8 d, p < 0.05). Ten (32.3%) children in IKD group presented with bilateral conjunctival congestion. WBC and alanine transaminase (ALT) in IKD group were lower in IKD group. No differences were identified in other laboratory study results between two groups. The rate of non-responders of IVIG in IKD group is lower than KD group (3.3% vs 18.3%). Coronary artery lesions in IKD include coronary dilatation in 22(71%), aneurysm in 5 (16.1%) and giant aneurysm in 3 (9.7%), which is higher than KD group (27.7%, 5.2% and 1.1%).

Conclusions Children with incomplete KD and cervical lymphadenopathy and fever as initial manifestations presented with longer period of fever and higher incidence of coronary artery aneurysm. Thus awareness of IKD should be kept in mind in this special group of children.