Dilaporkan sebuah kasus histiositosis sel Langerhans (HSL) tipe Hand-Schuller-Christian pada seorang anak laki-laki usia 3 tahun. Keluhan berupa penonjolan kedua bola mata sejak 4 bulan. Sejak usia 19 bulan terdapat bintil-bintil berwarna merah yang hilang timbul, gatal, dan bila sembuh meninggalkan bercak berwarna putih. Diagnosis ditegakkan berdasarkan anamnesis, gambaran klinis, pemeriksaan laboratorium, radiologis dan histopatologik. Pasien dirawat di bagian anak, diberikan terapi suportif, dan rencana akan diberikan kemoterapi.

Kata kunci: histiositosis sel Langerhans, Hand-Schuller-Christian, kemoterapi.

    A case of Langerhans cell histiocytosis (LCH) Hand-Schuller-Christian in a 3-year old boy was reported. The chief complaint  was protrusion of the eyes since 4 months ago. It followed by recurrent itchy skin lesions consist of erythematous papules, which healed and leave persistent hypopigmentation areas since he was 19 months. The diagnosis was confirmed by anamnesis, clinical appearances, laboratoric exam, radiologic exam and histopathologic examinations. The patient was in pediatric ward. He was given suportive therapies, and prepare for the chemotherapy

Key words: Langerhans cell histiocytosis, Hand-Schuller-Christian, chemoterapy.