Background: Chromoblastomycosis (CBM) is a chronic subcutaneous mycosis caused by dematiaceous fungi. Methods: We described epidemiological data, clinical presentation, and treatment of 18 cases of CBM diagnosed in Rio de Janeiro, Brazil. Diagnosis was obtained by mycological, histopathological findings demonstrating typical muriform cells with confirmation of isolated by DNA sequencing of the ribosomal internal transcribed spacer. Results: The majority of patients were male (72.2%) ranging from 39 to 83 years old, farm laborers and construction workers. The duration of disease varied from four months to 32 years. The most common presentations were verrucous form in ten (55.6%) patients, followed by tumoral in three (16.7%) patients, primarily of moderate (55.6%) and severe (38.9%) intensity. Lower (44.4%) and upper limbs (33.3%) were the most affected sites. Fonsecaea pedrosoi isolated from 14 (77.8%), and Cladophialophora carrionii isolated from one case (5.6%). Fifteen patients (83.3%) were treated. Six patients (40%) received oral itraconazole 200–400 mg/day, five patients (33.3%) received oral itraconazole 200–400 mg/day combined with fluconazole 200 mg/day, and four (26.7%) patients were submitted to surgery. The duration of therapy varied from 12 to 48 months. Cure rate was 80% (12/15). No relapse was observed after two years of follow-up. Conclusions: Success was due to attending a center with specialized clinical care, laboratory support, and pharmaceutical care.