Lipoblastoma/lipoblastomatosis is an uncommon benign lipomatous tumor affecting mainly infants and children. It presents as a painless mass located frequently in upper and lower extremities. The present study was conducted to characterize the clinicopathologic features of lipoblastoma/lipoblastomatosis in a Chinese population. A retrospective survey of 16 cases of pathologically confirmed lipoblastoma/lipoblastomatosis from 1988 to 2002 was performed. The available clinical information and histological sections were reviewed. The study involved 10 males and 6 females ranging in age from 5 to 49 months (median, 11.5 months). The patients presented with mass involving neck (n=5), inguina (n=3), feet (n=2), arm (n=1), leg (n=1), thorax (n=1), mesentery (n=1), buttock (n=1), and presacral region(n=1). The lesions were removed surgically. Histologically, the majority (11/16) of primary tumors were of diffuse type. Follow-up ranging from 5 to 125 months was available in 13 patients. Four patients experienced a recurrence of tumors in the neck (n=2) and lower extremities (n=2) 11–84 months after operation. The most common site of involvement of lipoblastoma/lipoblastomatosis in our series was the neck. A follow-up period of more than 3 years is recommended for patients with this condition.