Abstract: Objective: To analyze clinical and histopathological features of connective tissue nevi in children. Methods: Clinical data were collected of connective tissue nevi in Capital Institute of Pediatrics from March 2018 to March 2022, and analyzed retrospectively. Results: The data of 7 children were analyzed, including 5 males and 2 females. The average age at the clinic visit was 6.7 years. The average onset age was 2.8 years. The average duration of lesion was 4 years. Four children's lesions were on the thigh. The other 3 were respectively on the lumbar region, buttock, lumbar region and bottock. The clinical manifestation of the lesions were plaques, 4 were pigmented, 3 were depigmented. Except 1 child was diagnosed with tuberous sclerosis, the 6 children were without other organ involvement. There were 6 collagen type, and the child with tuberous sclerosis refused the test. Six children were acquired type, 1 was hereditary type. Conclusion: The average onset age of children with connective tissue nevi was as early as 2.8 years old. It is common in the lumbosacral and thigh, presenting as tough plaques. It may be related to the early diagnosis and short course of the disease, the skin lesions did not develop into obvious nodules. Very few have family history, and most of them are acquired type. They were mainly collagen type. The CTN children with othe organ involvement need to be ruled out certain syndrome.

Key words: connective tissue nevi, childhood, clinical features, histopathological features