Abstract: Zhang Xiaoyan, Wang Tingting, Wang Lin Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu 610041, China Corresponding author: Wang Lin, Email: lkzwl@126.com 【Abstract】 Objective To investigate clinicopathological features, diagnosis and treatment of progressive osseous heteroplasia （POH）. Methods Clinicopathological data were collected from 3 cases of POH, and analyzed retrospectively. Related literature was also reviewed. Results All the 3 patients were female, and developed POH within a few days to months after birth. POH clinically manifested as light pink or skin-colored indurated papules, nodules or plaques scattered over the scalp, trunk and extremities without subjective symptoms. Histopathological examination showed that there were flaky bone or bone-like tissues in the middle and lower dermis. Serum levels of calcium, phosphate and parathyroid hormone were normal in the 3 children. Case 1 was treated with oral sodium bicarbonate tablets and surgical resection of skin lesions on the right lower abdomen. During the follow-up for about 7 months, the skin lesions on the right lower abdomen did not recur, while other skin lesions became more bulging, larger and harder, and new skin lesions occurred. Case 2 was treated with topical adapalene gel, and these skin lesions did not increase in number, but became larger and harder. Case 3 received no treatment, and was lost to follow-up. Conclusion POH clinically manifests as indurated maculopapules and plaques progressively affecting deeper skin tissues, and there are no effective therapies or prevention approaches at present.